IMPDH1

UniProt ID: P20839
Organism: Homo sapiens
Review Status: INITIALIZED
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Gene Description

Inosine-5'-monophosphate dehydrogenase 1 (IMPDH1; EC 1.1.1.205) catalyses the NAD+-dependent oxidation of inosine 5'-monophosphate (IMP) to xanthosine 5'-monophosphate (XMP) (IMP + NAD+ + H2O -> XMP + NADH + H+), the first committed and rate-limiting step of de novo guanine-nucleotide (GMP) biosynthesis; XMP is subsequently aminated to GMP by GMP synthase (GMPS). The enzyme is a cytosolic homotetramer built from a catalytic (beta/alpha)8 TIM-barrel domain plus a regulatory subdomain of two tandem CBS (Bateman) domains, uses a monovalent cation (K+) cofactor, and can assemble into higher-order filaments ("rods and rings"/cytoophidia). IMPDH1 is broadly expressed but strongly enriched in retinal photoreceptors; a reported single-stranded nucleic-acid-binding activity mediated by the CBS subdomain suggests an additional, less well-defined role in nucleic-acid metabolism. Because guanine-nucleotide supply is coupled to cell proliferation, IMPDH is a validated target of immunosuppressive (mycophenolic acid, mizoribine), antiviral (ribavirin) and antitumour drugs. Dominant IMPDH1 mutations, many clustered in the CBS subdomain, cause retinitis pigmentosa 10 (RP10) and Leber congenital amaurosis (LCA11), degenerative diseases of the retina.

Existing Annotations Review

GO Term Evidence Action Reason
GO:0005737 cytoplasm
IBA
GO_REF:0000033
ACCEPT
Summary: Phylogenetically inferred cytoplasmic localization. IMPDH1 is a soluble cytosolic enzyme, consistent with its role in the cytosolic de novo purine pathway.
Reason: IBA is_active_in cytoplasm is correct and consistent with the UniProt subcellular location and with the more specific IDA cytosol annotation. This is a core localization for the enzyme's catalytic activity.
Supporting Evidence:
file:human/IMPDH1/IMPDH1-uniprot.txt
SUBCELLULAR LOCATION: Cytoplasm
GO:0003938 IMP dehydrogenase activity
IBA
GO_REF:0000033
ACCEPT
Summary: IMP dehydrogenase activity (EC 1.1.1.205) is the defining, experimentally and phylogenetically well-supported molecular function of IMPDH1: NAD+-dependent oxidation of IMP to XMP.
Reason: This is the core molecular function. Supported by the enzyme name, EC number, catalytic-activity reaction in UniProt, and by direct biochemical characterization of human type I IMPDH; the IBA is at the correct level of specificity.
Supporting Evidence:
file:human/IMPDH1/IMPDH1-uniprot.txt
Reaction=IMP + NAD(+) + H2O = XMP + NADH + H(+)
PMID:1969416
IMP dehydrogenase (EC 1.1.1.205), the rate-limiting enzyme of de novo GTP
GO:0006183 GTP biosynthetic process
IBA
GO_REF:0000033
KEEP AS NON CORE
Summary: IMPDH1 contributes to GTP biosynthesis only indirectly: it makes XMP (->GMP), and GMP is subsequently phosphorylated (GMP->GDP->GTP) by other enzymes.
Reason: The direct committed step catalysed by IMPDH1 produces XMP en route to GMP, not GTP. GTP biosynthesis is several enzymatic steps downstream, so this term overstates the enzyme's direct role. It is biologically defensible as a broader pathway contribution but is not the core function; the more precise BP terms are XMP/GMP biosynthetic process.
Supporting Evidence:
PMID:1969416
IMP dehydrogenase (EC 1.1.1.205), the rate-limiting enzyme of de novo GTP
GO:0000166 nucleotide binding
IEA
GO_REF:0000104
MODIFY
Summary: IMPDH1 binds the nucleotide cofactor NAD+ and the nucleotide substrate IMP; a generic "nucleotide binding" annotation captures this but at a very shallow level.
Reason: The term is not wrong but is uninformative given that the specific ligands (NAD+, IMP) and the catalytic activity are known. Replace with the specific cofactor-binding molecular function NAD binding (GO:0051287); the catalytic activity term already covers IMP as substrate.
Proposed replacements: NAD binding
Supporting Evidence:
file:human/IMPDH1/IMPDH1-uniprot.txt
Reaction=IMP + NAD(+) + H2O = XMP + NADH + H(+)
GO:0003824 catalytic activity
IEA
GO_REF:0000002
MODIFY
Summary: Root-level catalytic activity term derived from an InterPro domain mapping. IMPDH1 is an enzyme, so this is true but maximally uninformative.
Reason: "catalytic activity" is the top of the MF catalytic branch and adds nothing beyond the specific, well-established IMP dehydrogenase activity. Replace with GO:0003938 (IMP dehydrogenase activity).
Proposed replacements: IMP dehydrogenase activity
Supporting Evidence:
file:human/IMPDH1/IMPDH1-uniprot.txt
Reaction=IMP + NAD(+) + H2O = XMP + NADH + H(+)
GO:0003938 IMP dehydrogenase activity
IEA
GO_REF:0000120
ACCEPT
Summary: Electronic (UniRule/RHEA/EC-based) assertion of the core IMP dehydrogenase activity, duplicating the IBA and TAS annotations of the same term.
Reason: Correct and specific core molecular function; the IEA mapping from EC 1.1.1.205 / RHEA:11708 is well grounded. Duplicate of GO:0003938 by other evidence codes, which is acceptable.
Supporting Evidence:
file:human/IMPDH1/IMPDH1-uniprot.txt
Reaction=IMP + NAD(+) + H2O = XMP + NADH + H(+)
GO:0005634 nucleus
IEA
GO_REF:0000120
KEEP AS NON CORE
Summary: Nuclear localization transferred electronically, ultimately reflecting the reported nuclear pool of IMPDH linked to its nucleic-acid-binding activity.
Reason: IMPDH is detected in the nucleus (PMID:14766016), but the enzyme's core catalytic role in de novo GMP synthesis operates in the cytosol. Nuclear localization relates to the moonlighting single-stranded nucleic-acid-binding activity and is a non-core feature.
Supporting Evidence:
PMID:14766016
IMPDH is found in the nucleus of human cells, as
GO:0005737 cytoplasm
IEA
GO_REF:0000120
ACCEPT
Summary: Electronic cytoplasmic localization, consistent with the enzyme's soluble cytosolic distribution.
Reason: Correct core localization; duplicates the IBA/IDA cytoplasm and IDA cytosol annotations. The cytosol is where IMPDH1 performs its catalytic function.
Supporting Evidence:
file:human/IMPDH1/IMPDH1-uniprot.txt
SUBCELLULAR LOCATION: Cytoplasm
GO:0006164 purine nucleotide biosynthetic process
IEA
GO_REF:0000002
MODIFY
Summary: Broad "purine nucleotide biosynthetic process" from an InterPro mapping. IMPDH1 does act in de novo purine (guanine) nucleotide biosynthesis, but this is a high-level parent term.
Reason: Correct branch but too general; the specific, well-defined role is in guanosine (GMP)/XMP biosynthesis. Replace with the more precise GMP biosynthetic process (GO:0006177), which is the committed step catalysed by IMPDH1.
Proposed replacements: GMP biosynthetic process
Supporting Evidence:
file:human/IMPDH1/IMPDH1-uniprot.txt
step in the de novo synthesis of guanine nucleotides
GO:0006177 GMP biosynthetic process
IEA
GO_REF:0000120
ACCEPT
Summary: GMP biosynthetic process, the pathway in which IMPDH1 catalyses the committed, rate-limiting step (IMP -> XMP -> GMP).
Reason: This is the correct, appropriately specific core biological process for IMPDH1. The enzyme makes XMP, the immediate committed precursor of GMP, in the de novo guanine-nucleotide pathway.
Supporting Evidence:
file:human/IMPDH1/IMPDH1-uniprot.txt
step in the de novo synthesis of guanine nucleotides
GO:0016491 oxidoreductase activity
IEA
GO_REF:0000002
MODIFY
Summary: Parent oxidoreductase activity from an InterPro mapping. IMPDH1 is an NAD+-dependent oxidoreductase, but this is a broad ancestor of its specific activity.
Reason: True but too general; the specific dehydrogenase activity is IMP dehydrogenase activity (GO:0003938), a descendant of oxidoreductase activity. Replace with the specific term.
Proposed replacements: IMP dehydrogenase activity
Supporting Evidence:
file:human/IMPDH1/IMPDH1-uniprot.txt
Reaction=IMP + NAD(+) + H2O = XMP + NADH + H(+)
GO:0046872 metal ion binding
IEA
GO_REF:0000104
MODIFY
Summary: Metal-ion binding assigned by UniRule. IMPDH1 requires a monovalent cation (K+) cofactor, with several structurally defined K+ binding sites at the tetramer interface.
Reason: The generic "metal ion binding" understates the specific, functionally important requirement for a monovalent cation (potassium). Replace with potassium ion binding (GO:0030955), which the UniProt COFACTOR and K+ BINDING site annotations support.
Proposed replacements: potassium ion binding
Supporting Evidence:
file:human/IMPDH1/IMPDH1-uniprot.txt
Name=K(+)
GO:0097294 'de novo' XMP biosynthetic process
IEA
GO_REF:0000107
ACCEPT
Summary: 'de novo' XMP biosynthetic process is the most precise biological process for the reaction IMPDH1 actually catalyses: IMP -> XMP is the single committed step of de novo XMP synthesis.
Reason: Directly matches the UniProt pathway annotation ("XMP biosynthesis via de novo pathway; XMP from IMP: step 1/1") and the catalytic reaction. This is arguably the single most accurate BP term for IMPDH1 and a core process.
Supporting Evidence:
file:human/IMPDH1/IMPDH1-uniprot.txt
XMP biosynthesis via de novo pathway
GO:0005829 cytosol
IDA
GO_REF:0000052
ACCEPT
Summary: Direct immunofluorescence localization (HPA) to the cytosol, the compartment where IMPDH1 carries out de novo GMP synthesis.
Reason: Experimental (IDA) cytosolic localization is the most specific and accurate core location, consistent with the soluble cytosolic purine pathway and the broader cytoplasm annotations.
Supporting Evidence:
file:human/IMPDH1/IMPDH1-uniprot.txt
SUBCELLULAR LOCATION: Cytoplasm
GO:0005576 extracellular region
TAS
Reactome:R-HSA-6798748
MARK AS OVER ANNOTATED
Summary: Extracellular localization inferred from a Reactome neutrophil-degranulation exocytosis event (secretory granule proteins), not from a dedicated function of IMPDH1.
Reason: IMPDH1 is a soluble cytosolic enzyme. Its appearance in the extracellular region derives from bulk proteomic detection of the neutrophil granule/secretome and does not reflect a genuine secreted function. This is a bystander over-annotation, not a core localization.
Supporting Evidence:
file:human/IMPDH1/IMPDH1-uniprot.txt
SUBCELLULAR LOCATION: Cytoplasm
GO:0005576 extracellular region
TAS
Reactome:R-HSA-6798751
MARK AS OVER ANNOTATED
Summary: Duplicate extracellular-region localization from a Reactome azurophil-granule exocytosis event.
Reason: Same rationale as the other extracellular-region Reactome annotations: IMPDH1 is cytosolic, and its granule-lumen/extracellular assignment reflects neutrophil-degranulation proteomics rather than a genuine secreted role.
Supporting Evidence:
file:human/IMPDH1/IMPDH1-uniprot.txt
SUBCELLULAR LOCATION: Cytoplasm
GO:0005576 extracellular region
TAS
Reactome:R-HSA-6800434
MARK AS OVER ANNOTATED
Summary: Duplicate extracellular-region localization from a Reactome ficolin-rich granule exocytosis event.
Reason: As with the other Reactome degranulation-derived localizations, this reflects bulk granule/secretome proteomics rather than a genuine extracellular function of the cytosolic enzyme IMPDH1.
Supporting Evidence:
file:human/IMPDH1/IMPDH1-uniprot.txt
SUBCELLULAR LOCATION: Cytoplasm
GO:0034774 secretory granule lumen
TAS
Reactome:R-HSA-6798748
MARK AS OVER ANNOTATED
Summary: Secretory granule lumen localization from the Reactome neutrophil-degranulation pathway.
Reason: Cytosolic IMPDH1 is detected in neutrophil granule proteomes, but this is a bystander/contaminant-type localization from degranulation datasets rather than a genuine granule-lumen residence relevant to its enzymatic function.
Supporting Evidence:
file:human/IMPDH1/IMPDH1-uniprot.txt
SUBCELLULAR LOCATION: Cytoplasm
GO:0035578 azurophil granule lumen
TAS
Reactome:R-HSA-6798751
MARK AS OVER ANNOTATED
Summary: Azurophil granule lumen localization from the Reactome neutrophil-degranulation pathway.
Reason: As above: derives from neutrophil granule proteomics of the cytosolic enzyme IMPDH1 and does not reflect a core, functionally meaningful granule-lumen localization.
Supporting Evidence:
file:human/IMPDH1/IMPDH1-uniprot.txt
SUBCELLULAR LOCATION: Cytoplasm
GO:1904813 ficolin-1-rich granule lumen
TAS
Reactome:R-HSA-6800434
MARK AS OVER ANNOTATED
Summary: Ficolin-1-rich granule lumen localization from the Reactome neutrophil-degranulation pathway.
Reason: Same bystander-proteomics rationale: cytosolic IMPDH1 appearing in a granule lumen dataset does not constitute a genuine, core subcellular location for the enzyme.
Supporting Evidence:
file:human/IMPDH1/IMPDH1-uniprot.txt
SUBCELLULAR LOCATION: Cytoplasm
GO:0005829 cytosol
TAS
Reactome:R-HSA-73794
ACCEPT
Summary: Cytosolic localization asserted by the Reactome IMP -> XMP reaction, the core catalytic event of IMPDH1.
Reason: Correct core localization tied directly to the catalytic reaction; consistent with the IDA cytosol and cytoplasm annotations.
Supporting Evidence:
Reactome:R-HSA-73794
IMP dehydrogenase 1 and 2 (IMPDH1,2) catalyze the irreversible dehydrogenation of inosine 5'-monophosphate (IMP) to form xanthosine 5'-monophosphate (XMP)
GO:0005829 cytosol
TAS
Reactome:R-HSA-9678749
ACCEPT
Summary: Cytosolic localization from the Reactome "IMPDH tetramers bind IMPDH inhibitors" reaction (drug-binding context).
Reason: The cytosol is the correct core compartment for IMPDH1; the annotation arises from a drug/inhibitor-binding reaction but the localization itself is accurate and duplicates the other cytosol annotations.
Supporting Evidence:
file:human/IMPDH1/IMPDH1-uniprot.txt
SUBCELLULAR LOCATION: Cytoplasm
GO:0005829 cytosol
TAS
Reactome:R-HSA-9748945
ACCEPT
Summary: Cytosolic localization from the Reactome "IMPDH tetramers dehydrogenate 6TIMP to 6TXMP" reaction (thiopurine metabolism context).
Reason: Correct core localization. The reaction reflects IMPDH turnover of a thiopurine metabolite, but the cytosolic assignment is accurate and duplicates the other cytosol annotations.
Supporting Evidence:
file:human/IMPDH1/IMPDH1-uniprot.txt
SUBCELLULAR LOCATION: Cytoplasm
GO:0003676 nucleic acid binding
IDA
PMID:14766016
Inosine 5'-monophosphate dehydrogenase binds nucleic acids i...
KEEP AS NON CORE
Summary: Direct demonstration that IMPDH (both human isoforms) binds single-stranded nucleic acids with nanomolar affinity via its CBS subdomain, with ~100 nucleotides bound per tetramer.
Reason: This is a genuine, experimentally supported moonlighting activity of IMPDH1 mediated by the regulatory CBS (Bateman) subdomain, and several RP10/LCA disease variants alter this nucleic-acid affinity. However, the enzyme's core function is IMP dehydrogenase catalysis; the nucleic-acid-binding role is regulatory/moonlighting and best retained as non-core.
Supporting Evidence:
PMID:14766016
bind single-stranded nucleic acids with nanomolar affinity via the
GO:0003677 DNA binding
IDA
PMID:14766016
Inosine 5'-monophosphate dehydrogenase binds nucleic acids i...
KEEP AS NON CORE
Summary: IMPDH was shown by immunoprecipitation to bind DNA (and RNA) in vivo, an extension of its single-stranded nucleic-acid-binding activity.
Reason: Experimentally supported (IDA) but represents the moonlighting nucleic-acid interaction rather than a sequence-specific transcription-factor-type DNA binding. It is a non-core, regulatory activity distinct from the enzyme's committed metabolic role; retained as non-core per curation policy for experimental annotations.
Supporting Evidence:
PMID:14766016
immunoprecipitation experiments show that IMPDH binds both RNA and DNA in vivo
GO:0005634 nucleus
IDA
PMID:14766016
Inosine 5'-monophosphate dehydrogenase binds nucleic acids i...
KEEP AS NON CORE
Summary: Direct detection of IMPDH in the nucleus of human cells, consistent with its nucleic-acid-binding activity.
Reason: Experimentally supported nuclear pool, but the enzyme's core catalytic function in de novo GMP synthesis is cytosolic. Nuclear localization is tied to the moonlighting nucleic-acid-binding role and is non-core.
Supporting Evidence:
PMID:14766016
IMPDH is found in the nucleus of human cells, as
GO:0005737 cytoplasm
IDA
PMID:14766016
Inosine 5'-monophosphate dehydrogenase binds nucleic acids i...
ACCEPT
Summary: Direct localization of IMPDH to the cytoplasm, its principal compartment.
Reason: Experimental (IDA) cytoplasmic localization corroborates the core cytosolic residence where IMPDH1 catalyses IMP -> XMP; consistent with the IDA cytosol and IBA/IEA cytoplasm annotations.
Supporting Evidence:
file:human/IMPDH1/IMPDH1-uniprot.txt
SUBCELLULAR LOCATION: Cytoplasm
GO:0003938 IMP dehydrogenase activity
TAS
PMID:1969416
Two distinct cDNAs for human IMP dehydrogenase.
ACCEPT
Summary: Traceable author statement (original cloning paper) that the two human IMPDH cDNAs encode IMP dehydrogenase, the rate-limiting enzyme of de novo guanine nucleotide synthesis.
Reason: Core molecular function supported by the foundational cloning/characterization paper for human IMPDH1 (type I). Duplicates GO:0003938 by other evidence codes, which is acceptable.
Supporting Evidence:
PMID:1969416
IMP dehydrogenase (EC 1.1.1.205), the rate-limiting enzyme of de novo GTP

Core Functions

NAD+-dependent oxidation of inosine 5'-monophosphate (IMP) to xanthosine 5'-monophosphate (XMP), the committed and rate-limiting step of de novo guanine-nucleotide biosynthesis, performed in the cytosol.

Molecular Function:
IMP dehydrogenase activity
Cellular Locations:
Supporting Evidence:
  • file:human/IMPDH1/IMPDH1-uniprot.txt
    Reaction=IMP + NAD(+) + H2O = XMP + NADH + H(+)
  • PMID:1969416
    IMP dehydrogenase (EC 1.1.1.205), the rate-limiting enzyme of de novo GTP

Committed step of de novo GMP biosynthesis: by producing XMP, IMPDH1 supplies the immediate precursor that GMP synthase aminates to GMP, thereby providing guanine nucleotides for the cell.

Molecular Function:
IMP dehydrogenase activity
Directly Involved In:
Cellular Locations:
Supporting Evidence:
  • file:human/IMPDH1/IMPDH1-uniprot.txt
    step in the de novo synthesis of guanine nucleotides

References

Gene Ontology annotation through association of InterPro records with GO terms
Annotation inferences using phylogenetic trees
Gene Ontology annotation based on curation of immunofluorescence data
Electronic Gene Ontology annotations created by transferring manual GO annotations between related proteins based on shared sequence features
Automatic transfer of experimentally verified manual GO annotation data to orthologs using Ensembl Compara
Combined Automated Annotation using Multiple IEA Methods
Inosine 5'-monophosphate dehydrogenase binds nucleic acids in vitro and in vivo.
Two distinct cDNAs for human IMP dehydrogenase.
Reactome:R-HSA-6798748
Exocytosis of secretory granule lumen proteins
Reactome:R-HSA-6798751
Exocytosis of azurophil granule lumen proteins
Reactome:R-HSA-6800434
Exocytosis of ficolin-rich granule lumen proteins
Reactome:R-HSA-73794
IMP + H2O + NAD+ => XMP + NADH + H+ [IMPDH1,2]
Reactome:R-HSA-9678749
IMPDH tetramers bind IMPDH inhibitors
Reactome:R-HSA-9748945
IMPDH tetramers dehydrogenate 6TIMP to 6TXMP
file:human/IMPDH1/IMPDH1-uniprot.txt
UniProtKB P20839 (IMDH1_HUMAN) curated entry

Suggested Questions for Experts

Q: Is the single-stranded nucleic-acid-binding activity of the CBS subdomain a physiologically regulated function of IMPDH1 in photoreceptors, and does its disruption (as by RP10/LCA CBS-domain variants) cause disease independently of catalytic activity?

Q: What is the functional significance of IMPDH1 filament ("rods and rings"/ cytoophidia) assembly for guanine-nucleotide homeostasis in the retina?

Suggested Experiments

Experiment: Separate-of-function IMPDH1 alleles that abolish catalysis while preserving nucleic-acid binding (and vice versa) expressed in photoreceptor models, to test whether RP10/LCA phenotypes arise from loss of catalysis, loss/alteration of nucleic-acid binding, or a dominant-negative filament defect.

Experiment: Cryo-EM and cellular imaging of retina-specific IMPDH1 isoforms to determine how the extended N-terminal retinal isoforms modulate filament assembly and GTP/GDP-dependent allosteric regulation.

📚 Additional Documentation

Notes

(IMPDH1-notes.md)

IMPDH1 (human, P20839) review notes

Verified biology (grounded in UniProt P20839, GOA, cached PMIDs, Reactome)

IMPDH1 = inosine-5'-monophosphate dehydrogenase 1 (EC 1.1.1.205). Catalyzes the
committed, rate-limiting step of de novo guanine-nucleotide synthesis:
IMP + NAD+ + H2O -> XMP + NADH + H+ (RHEA:11708). XMP is then aminated to GMP by
GMPS. UniProt FUNCTION: "Catalyzes the conversion of inosine 5'-phosphate (IMP) to
xanthosine 5'-phosphate (XMP), the first committed and rate-limiting step in the de
novo synthesis of guanine nucleotides".

  • Homotetramer (UniProt SUBUNIT; PubMed:7903306). Contains catalytic (β/α)8 TIM barrel
  • a regulatory subdomain of two CBS (Bateman) domains (FT DOMAIN 114-173 CBS 1,
    179-237 CBS 2). Can polymerize into filaments ("rods and rings"/cytoophidia) — many
    cryo-EM structures (7RER..8U8Y).
  • Cofactor K+ (UniProt COFACTOR; multiple K+ BINDING sites). Uses NAD+ (BINDING 274-276,
    324-326). Active sites: Cys331 (thioimidate intermediate), 429 (proton acceptor).
  • Subcellular: Cytoplasm + Nucleus (UniProt; PubMed:14766016). HPA IDA cytosol.
  • Retina-enriched (UniProt HPA: "Tissue enhanced (retina)").
  • Disease: Retinitis pigmentosa 10 (RP10, MIM:180105) and Leber congenital amaurosis 11
    (LCA11, MIM:613837) — photoreceptor degeneration. Many disease variants map to the CBS
    subdomain and alter single-stranded nucleic-acid affinity but NOT enzymatic affinity
    (PubMed:16384941 variant notes).

Moonlighting / non-core: nucleic-acid binding

PMID:14766016 (McLean et al. 2004, abstract only): IMPDHs (T. foetus, E. coli, both human
isoforms) bind single-stranded nucleic acids with nanomolar affinity via the CBS subdomain;
~100 nt per tetramer. IMPDH found in nucleus; immunoprecipitation shows binding of RNA and
DNA in vivo. Concluded "a previously unappreciated role in replication, transcription or
translation." This is a moonlighting/regulatory activity, NOT the core catalytic function.
UniProt hedges: "Could also have a single-stranded nucleic acid-binding activity and could
play a role in RNA and/or DNA metabolism." => treat DNA/nucleic-acid binding and the nucleus
localization associated with it as KEEP_AS_NON_CORE.

GOA annotations summary (from IMPDH1-goa.tsv)

Core MF: GO:0003938 IMP dehydrogenase activity (IBA, IEA, TAS PMID:1969416).
Core BP: GO:0006177 GMP biosynthetic process (IEA); GO:0097294 'de novo' XMP biosynthetic
process (IEA/Ensembl orthology) — most precise BP for the actual reaction; GO:0006183 GTP
biosynthetic process (IBA) — one step removed (XMP->GMP->GDP->GTP), keep as non-core;
GO:0006164 purine nucleotide biosynthetic process (IEA, broad parent).
CC: GO:0005829 cytosol (IDA HPA; TAS Reactome) — core location; GO:0005737 cytoplasm
(IBA is_active_in, IEA, IDA) — core; GO:0005634 nucleus (IEA, IDA PMID:14766016) — non-core
moonlighting. Extracellular/granule-lumen (Reactome neutrophil degranulation) = bystander
secretome, non-core.

Reactome refs

R-HSA-73794 is the actual IMP->XMP reaction (core). R-HSA-9678749/9748945 concern IMPDH
inhibitor binding / thiopurine (6TIMP) turnover (drug-metabolism context). R-HSA-6798748/
6798751/6800434 are neutrophil-degranulation exocytosis events (extracellular/granule lumen
localization by mass-spec proteomics of granules) — bystander, non-core.

📄 View Raw YAML

id: P20839
gene_symbol: IMPDH1
product_type: PROTEIN
status: INITIALIZED
taxon:
  id: NCBITaxon:9606
  label: Homo sapiens
description: >-
  Inosine-5'-monophosphate dehydrogenase 1 (IMPDH1; EC 1.1.1.205) catalyses the
  NAD+-dependent oxidation of inosine 5'-monophosphate (IMP) to xanthosine
  5'-monophosphate (XMP) (IMP + NAD+ + H2O -> XMP + NADH + H+), the first
  committed and rate-limiting step of de novo guanine-nucleotide (GMP)
  biosynthesis; XMP is subsequently aminated to GMP by GMP synthase (GMPS). The
  enzyme is a cytosolic homotetramer built from a catalytic (beta/alpha)8 TIM-barrel
  domain plus a regulatory subdomain of two tandem CBS (Bateman) domains, uses a
  monovalent cation (K+) cofactor, and can assemble into higher-order filaments
  ("rods and rings"/cytoophidia). IMPDH1 is broadly expressed but strongly enriched
  in retinal photoreceptors; a reported single-stranded nucleic-acid-binding
  activity mediated by the CBS subdomain suggests an additional, less well-defined
  role in nucleic-acid metabolism. Because guanine-nucleotide supply is coupled to
  cell proliferation, IMPDH is a validated target of immunosuppressive (mycophenolic
  acid, mizoribine), antiviral (ribavirin) and antitumour drugs. Dominant IMPDH1
  mutations, many clustered in the CBS subdomain, cause retinitis pigmentosa 10
  (RP10) and Leber congenital amaurosis (LCA11), degenerative diseases of the retina.
references:
- id: GO_REF:0000002
  title: Gene Ontology annotation through association of InterPro records with GO
    terms
  findings: []
- id: GO_REF:0000033
  title: Annotation inferences using phylogenetic trees
  findings: []
- id: GO_REF:0000052
  title: Gene Ontology annotation based on curation of immunofluorescence data
  findings: []
- id: GO_REF:0000104
  title: Electronic Gene Ontology annotations created by transferring manual GO annotations
    between related proteins based on shared sequence features
  findings: []
- id: GO_REF:0000107
  title: Automatic transfer of experimentally verified manual GO annotation data to
    orthologs using Ensembl Compara
  findings: []
- id: GO_REF:0000120
  title: Combined Automated Annotation using Multiple IEA Methods
  findings: []
- id: PMID:14766016
  title: Inosine 5'-monophosphate dehydrogenase binds nucleic acids in vitro and in
    vivo.
  findings: []
  reference_review:
    relevance: MEDIUM
    correctness: VERIFIED
    review_notes: >-
      PubMed-verified (Hedstrom lab, Biochem J 2004). Establishes the CBS-subdomain
      single-stranded nucleic-acid-binding activity for both human IMPDH isoforms
      and IMPDH nuclear localization. Supports a moonlighting/regulatory (non-core)
      role rather than the enzyme's principal catalytic function; abstract-only in
      the cache.
- id: PMID:1969416
  title: Two distinct cDNAs for human IMP dehydrogenase.
  findings: []
  reference_review:
    relevance: HIGH
    correctness: VERIFIED
    review_notes: >-
      PubMed-verified (Natsumeda et al., J Biol Chem 1990). Original cloning of the
      two human IMPDH cDNAs (type I = IMPDH1, type II = IMPDH2); identifies IMP
      dehydrogenase (EC 1.1.1.205) as the rate-limiting enzyme of de novo guanine
      nucleotide biosynthesis. Directly supports the core catalytic function.
- id: Reactome:R-HSA-6798748
  title: Exocytosis of secretory granule lumen proteins
  findings: []
- id: Reactome:R-HSA-6798751
  title: Exocytosis of azurophil granule lumen proteins
  findings: []
- id: Reactome:R-HSA-6800434
  title: Exocytosis of ficolin-rich granule lumen proteins
  findings: []
- id: Reactome:R-HSA-73794
  title: IMP + H2O + NAD+ => XMP + NADH + H+ [IMPDH1,2]
  findings: []
  reference_review:
    relevance: HIGH
    correctness: VERIFIED
    review_notes: >-
      Reactome reaction for the human IMP -> XMP dehydrogenation catalysed by IMPDH1
      and IMPDH2; the summary confirms this is the rate-limiting step in GMP synthesis
      from IMP. Directly supports the core molecular function.
- id: Reactome:R-HSA-9678749
  title: IMPDH tetramers bind IMPDH inhibitors
  findings: []
- id: Reactome:R-HSA-9748945
  title: IMPDH tetramers dehydrogenate 6TIMP to 6TXMP
  findings: []
- id: file:human/IMPDH1/IMPDH1-uniprot.txt
  title: UniProtKB P20839 (IMDH1_HUMAN) curated entry
  findings: []
existing_annotations:
- term:
    id: GO:0005737
    label: cytoplasm
  evidence_type: IBA
  original_reference_id: GO_REF:0000033
  qualifier: is_active_in
  review:
    summary: >-
      Phylogenetically inferred cytoplasmic localization. IMPDH1 is a soluble
      cytosolic enzyme, consistent with its role in the cytosolic de novo purine
      pathway.
    action: ACCEPT
    reason: >-
      IBA is_active_in cytoplasm is correct and consistent with the UniProt
      subcellular location and with the more specific IDA cytosol annotation. This
      is a core localization for the enzyme's catalytic activity.
    supported_by:
    - reference_id: file:human/IMPDH1/IMPDH1-uniprot.txt
      supporting_text: "SUBCELLULAR LOCATION: Cytoplasm"
- term:
    id: GO:0003938
    label: IMP dehydrogenase activity
  evidence_type: IBA
  original_reference_id: GO_REF:0000033
  qualifier: enables
  review:
    summary: >-
      IMP dehydrogenase activity (EC 1.1.1.205) is the defining, experimentally and
      phylogenetically well-supported molecular function of IMPDH1: NAD+-dependent
      oxidation of IMP to XMP.
    action: ACCEPT
    reason: >-
      This is the core molecular function. Supported by the enzyme name, EC number,
      catalytic-activity reaction in UniProt, and by direct biochemical
      characterization of human type I IMPDH; the IBA is at the correct level of
      specificity.
    supported_by:
    - reference_id: file:human/IMPDH1/IMPDH1-uniprot.txt
      supporting_text: "Reaction=IMP + NAD(+) + H2O = XMP + NADH + H(+)"
    - reference_id: PMID:1969416
      supporting_text: "IMP dehydrogenase (EC 1.1.1.205), the rate-limiting enzyme of de novo GTP"
- term:
    id: GO:0006183
    label: GTP biosynthetic process
  evidence_type: IBA
  original_reference_id: GO_REF:0000033
  qualifier: involved_in
  review:
    summary: >-
      IMPDH1 contributes to GTP biosynthesis only indirectly: it makes XMP (->GMP),
      and GMP is subsequently phosphorylated (GMP->GDP->GTP) by other enzymes.
    action: KEEP_AS_NON_CORE
    reason: >-
      The direct committed step catalysed by IMPDH1 produces XMP en route to GMP,
      not GTP. GTP biosynthesis is several enzymatic steps downstream, so this term
      overstates the enzyme's direct role. It is biologically defensible as a broader
      pathway contribution but is not the core function; the more precise BP terms are
      XMP/GMP biosynthetic process.
    supported_by:
    - reference_id: PMID:1969416
      supporting_text: "IMP dehydrogenase (EC 1.1.1.205), the rate-limiting enzyme of de novo GTP"
- term:
    id: GO:0000166
    label: nucleotide binding
  evidence_type: IEA
  original_reference_id: GO_REF:0000104
  qualifier: enables
  review:
    summary: >-
      IMPDH1 binds the nucleotide cofactor NAD+ and the nucleotide substrate IMP; a
      generic "nucleotide binding" annotation captures this but at a very shallow
      level.
    action: MODIFY
    reason: >-
      The term is not wrong but is uninformative given that the specific ligands
      (NAD+, IMP) and the catalytic activity are known. Replace with the specific
      cofactor-binding molecular function NAD binding (GO:0051287); the catalytic
      activity term already covers IMP as substrate.
    proposed_replacement_terms:
    - id: GO:0051287
      label: NAD binding
    supported_by:
    - reference_id: file:human/IMPDH1/IMPDH1-uniprot.txt
      supporting_text: "Reaction=IMP + NAD(+) + H2O = XMP + NADH + H(+)"
- term:
    id: GO:0003824
    label: catalytic activity
  evidence_type: IEA
  original_reference_id: GO_REF:0000002
  qualifier: enables
  review:
    summary: >-
      Root-level catalytic activity term derived from an InterPro domain mapping.
      IMPDH1 is an enzyme, so this is true but maximally uninformative.
    action: MODIFY
    reason: >-
      "catalytic activity" is the top of the MF catalytic branch and adds nothing
      beyond the specific, well-established IMP dehydrogenase activity. Replace with
      GO:0003938 (IMP dehydrogenase activity).
    proposed_replacement_terms:
    - id: GO:0003938
      label: IMP dehydrogenase activity
    supported_by:
    - reference_id: file:human/IMPDH1/IMPDH1-uniprot.txt
      supporting_text: "Reaction=IMP + NAD(+) + H2O = XMP + NADH + H(+)"
- term:
    id: GO:0003938
    label: IMP dehydrogenase activity
  evidence_type: IEA
  original_reference_id: GO_REF:0000120
  qualifier: enables
  review:
    summary: >-
      Electronic (UniRule/RHEA/EC-based) assertion of the core IMP dehydrogenase
      activity, duplicating the IBA and TAS annotations of the same term.
    action: ACCEPT
    reason: >-
      Correct and specific core molecular function; the IEA mapping from EC 1.1.1.205
      / RHEA:11708 is well grounded. Duplicate of GO:0003938 by other evidence codes,
      which is acceptable.
    supported_by:
    - reference_id: file:human/IMPDH1/IMPDH1-uniprot.txt
      supporting_text: "Reaction=IMP + NAD(+) + H2O = XMP + NADH + H(+)"
- term:
    id: GO:0005634
    label: nucleus
  evidence_type: IEA
  original_reference_id: GO_REF:0000120
  qualifier: located_in
  review:
    summary: >-
      Nuclear localization transferred electronically, ultimately reflecting the
      reported nuclear pool of IMPDH linked to its nucleic-acid-binding activity.
    action: KEEP_AS_NON_CORE
    reason: >-
      IMPDH is detected in the nucleus (PMID:14766016), but the enzyme's core
      catalytic role in de novo GMP synthesis operates in the cytosol. Nuclear
      localization relates to the moonlighting single-stranded nucleic-acid-binding
      activity and is a non-core feature.
    supported_by:
    - reference_id: PMID:14766016
      supporting_text: "IMPDH is found in the nucleus of human cells, as"
- term:
    id: GO:0005737
    label: cytoplasm
  evidence_type: IEA
  original_reference_id: GO_REF:0000120
  qualifier: located_in
  review:
    summary: >-
      Electronic cytoplasmic localization, consistent with the enzyme's soluble
      cytosolic distribution.
    action: ACCEPT
    reason: >-
      Correct core localization; duplicates the IBA/IDA cytoplasm and IDA cytosol
      annotations. The cytosol is where IMPDH1 performs its catalytic function.
    supported_by:
    - reference_id: file:human/IMPDH1/IMPDH1-uniprot.txt
      supporting_text: "SUBCELLULAR LOCATION: Cytoplasm"
- term:
    id: GO:0006164
    label: purine nucleotide biosynthetic process
  evidence_type: IEA
  original_reference_id: GO_REF:0000002
  qualifier: involved_in
  review:
    summary: >-
      Broad "purine nucleotide biosynthetic process" from an InterPro mapping.
      IMPDH1 does act in de novo purine (guanine) nucleotide biosynthesis, but this
      is a high-level parent term.
    action: MODIFY
    reason: >-
      Correct branch but too general; the specific, well-defined role is in guanosine
      (GMP)/XMP biosynthesis. Replace with the more precise GMP biosynthetic process
      (GO:0006177), which is the committed step catalysed by IMPDH1.
    proposed_replacement_terms:
    - id: GO:0006177
      label: GMP biosynthetic process
    supported_by:
    - reference_id: file:human/IMPDH1/IMPDH1-uniprot.txt
      supporting_text: "step in the de novo synthesis of guanine nucleotides"
- term:
    id: GO:0006177
    label: GMP biosynthetic process
  evidence_type: IEA
  original_reference_id: GO_REF:0000120
  qualifier: involved_in
  review:
    summary: >-
      GMP biosynthetic process, the pathway in which IMPDH1 catalyses the committed,
      rate-limiting step (IMP -> XMP -> GMP).
    action: ACCEPT
    reason: >-
      This is the correct, appropriately specific core biological process for
      IMPDH1. The enzyme makes XMP, the immediate committed precursor of GMP, in the
      de novo guanine-nucleotide pathway.
    supported_by:
    - reference_id: file:human/IMPDH1/IMPDH1-uniprot.txt
      supporting_text: "step in the de novo synthesis of guanine nucleotides"
- term:
    id: GO:0016491
    label: oxidoreductase activity
  evidence_type: IEA
  original_reference_id: GO_REF:0000002
  qualifier: enables
  review:
    summary: >-
      Parent oxidoreductase activity from an InterPro mapping. IMPDH1 is an
      NAD+-dependent oxidoreductase, but this is a broad ancestor of its specific
      activity.
    action: MODIFY
    reason: >-
      True but too general; the specific dehydrogenase activity is IMP dehydrogenase
      activity (GO:0003938), a descendant of oxidoreductase activity. Replace with
      the specific term.
    proposed_replacement_terms:
    - id: GO:0003938
      label: IMP dehydrogenase activity
    supported_by:
    - reference_id: file:human/IMPDH1/IMPDH1-uniprot.txt
      supporting_text: "Reaction=IMP + NAD(+) + H2O = XMP + NADH + H(+)"
- term:
    id: GO:0046872
    label: metal ion binding
  evidence_type: IEA
  original_reference_id: GO_REF:0000104
  qualifier: enables
  review:
    summary: >-
      Metal-ion binding assigned by UniRule. IMPDH1 requires a monovalent cation
      (K+) cofactor, with several structurally defined K+ binding sites at the
      tetramer interface.
    action: MODIFY
    reason: >-
      The generic "metal ion binding" understates the specific, functionally
      important requirement for a monovalent cation (potassium). Replace with
      potassium ion binding (GO:0030955), which the UniProt COFACTOR and K+ BINDING
      site annotations support.
    proposed_replacement_terms:
    - id: GO:0030955
      label: potassium ion binding
    supported_by:
    - reference_id: file:human/IMPDH1/IMPDH1-uniprot.txt
      supporting_text: "Name=K(+)"
- term:
    id: GO:0097294
    label: "'de novo' XMP biosynthetic process"
  evidence_type: IEA
  original_reference_id: GO_REF:0000107
  qualifier: involved_in
  review:
    summary: >-
      'de novo' XMP biosynthetic process is the most precise biological process for
      the reaction IMPDH1 actually catalyses: IMP -> XMP is the single committed step
      of de novo XMP synthesis.
    action: ACCEPT
    reason: >-
      Directly matches the UniProt pathway annotation ("XMP biosynthesis via de novo
      pathway; XMP from IMP: step 1/1") and the catalytic reaction. This is arguably
      the single most accurate BP term for IMPDH1 and a core process.
    supported_by:
    - reference_id: file:human/IMPDH1/IMPDH1-uniprot.txt
      supporting_text: "XMP biosynthesis via de novo pathway"
- term:
    id: GO:0005829
    label: cytosol
  evidence_type: IDA
  original_reference_id: GO_REF:0000052
  qualifier: located_in
  review:
    summary: >-
      Direct immunofluorescence localization (HPA) to the cytosol, the compartment
      where IMPDH1 carries out de novo GMP synthesis.
    action: ACCEPT
    reason: >-
      Experimental (IDA) cytosolic localization is the most specific and accurate
      core location, consistent with the soluble cytosolic purine pathway and the
      broader cytoplasm annotations.
    supported_by:
    - reference_id: file:human/IMPDH1/IMPDH1-uniprot.txt
      supporting_text: "SUBCELLULAR LOCATION: Cytoplasm"
- term:
    id: GO:0005576
    label: extracellular region
  evidence_type: TAS
  original_reference_id: Reactome:R-HSA-6798748
  qualifier: located_in
  review:
    summary: >-
      Extracellular localization inferred from a Reactome neutrophil-degranulation
      exocytosis event (secretory granule proteins), not from a dedicated function
      of IMPDH1.
    action: MARK_AS_OVER_ANNOTATED
    reason: >-
      IMPDH1 is a soluble cytosolic enzyme. Its appearance in the extracellular
      region derives from bulk proteomic detection of the neutrophil granule/secretome
      and does not reflect a genuine secreted function. This is a bystander
      over-annotation, not a core localization.
    supported_by:
    - reference_id: file:human/IMPDH1/IMPDH1-uniprot.txt
      supporting_text: "SUBCELLULAR LOCATION: Cytoplasm"
- term:
    id: GO:0005576
    label: extracellular region
  evidence_type: TAS
  original_reference_id: Reactome:R-HSA-6798751
  qualifier: located_in
  review:
    summary: >-
      Duplicate extracellular-region localization from a Reactome azurophil-granule
      exocytosis event.
    action: MARK_AS_OVER_ANNOTATED
    reason: >-
      Same rationale as the other extracellular-region Reactome annotations: IMPDH1
      is cytosolic, and its granule-lumen/extracellular assignment reflects
      neutrophil-degranulation proteomics rather than a genuine secreted role.
    supported_by:
    - reference_id: file:human/IMPDH1/IMPDH1-uniprot.txt
      supporting_text: "SUBCELLULAR LOCATION: Cytoplasm"
- term:
    id: GO:0005576
    label: extracellular region
  evidence_type: TAS
  original_reference_id: Reactome:R-HSA-6800434
  qualifier: located_in
  review:
    summary: >-
      Duplicate extracellular-region localization from a Reactome ficolin-rich
      granule exocytosis event.
    action: MARK_AS_OVER_ANNOTATED
    reason: >-
      As with the other Reactome degranulation-derived localizations, this reflects
      bulk granule/secretome proteomics rather than a genuine extracellular function
      of the cytosolic enzyme IMPDH1.
    supported_by:
    - reference_id: file:human/IMPDH1/IMPDH1-uniprot.txt
      supporting_text: "SUBCELLULAR LOCATION: Cytoplasm"
- term:
    id: GO:0034774
    label: secretory granule lumen
  evidence_type: TAS
  original_reference_id: Reactome:R-HSA-6798748
  qualifier: located_in
  review:
    summary: >-
      Secretory granule lumen localization from the Reactome neutrophil-degranulation
      pathway.
    action: MARK_AS_OVER_ANNOTATED
    reason: >-
      Cytosolic IMPDH1 is detected in neutrophil granule proteomes, but this is a
      bystander/contaminant-type localization from degranulation datasets rather than
      a genuine granule-lumen residence relevant to its enzymatic function.
    supported_by:
    - reference_id: file:human/IMPDH1/IMPDH1-uniprot.txt
      supporting_text: "SUBCELLULAR LOCATION: Cytoplasm"
- term:
    id: GO:0035578
    label: azurophil granule lumen
  evidence_type: TAS
  original_reference_id: Reactome:R-HSA-6798751
  qualifier: located_in
  review:
    summary: >-
      Azurophil granule lumen localization from the Reactome neutrophil-degranulation
      pathway.
    action: MARK_AS_OVER_ANNOTATED
    reason: >-
      As above: derives from neutrophil granule proteomics of the cytosolic enzyme
      IMPDH1 and does not reflect a core, functionally meaningful granule-lumen
      localization.
    supported_by:
    - reference_id: file:human/IMPDH1/IMPDH1-uniprot.txt
      supporting_text: "SUBCELLULAR LOCATION: Cytoplasm"
- term:
    id: GO:1904813
    label: ficolin-1-rich granule lumen
  evidence_type: TAS
  original_reference_id: Reactome:R-HSA-6800434
  qualifier: located_in
  review:
    summary: >-
      Ficolin-1-rich granule lumen localization from the Reactome
      neutrophil-degranulation pathway.
    action: MARK_AS_OVER_ANNOTATED
    reason: >-
      Same bystander-proteomics rationale: cytosolic IMPDH1 appearing in a granule
      lumen dataset does not constitute a genuine, core subcellular location for the
      enzyme.
    supported_by:
    - reference_id: file:human/IMPDH1/IMPDH1-uniprot.txt
      supporting_text: "SUBCELLULAR LOCATION: Cytoplasm"
- term:
    id: GO:0005829
    label: cytosol
  evidence_type: TAS
  original_reference_id: Reactome:R-HSA-73794
  qualifier: located_in
  review:
    summary: >-
      Cytosolic localization asserted by the Reactome IMP -> XMP reaction, the core
      catalytic event of IMPDH1.
    action: ACCEPT
    reason: >-
      Correct core localization tied directly to the catalytic reaction; consistent
      with the IDA cytosol and cytoplasm annotations.
    supported_by:
    - reference_id: Reactome:R-HSA-73794
      supporting_text: "IMP dehydrogenase 1 and 2 (IMPDH1,2) catalyze the irreversible dehydrogenation of inosine 5'-monophosphate (IMP) to form xanthosine 5'-monophosphate (XMP)"
- term:
    id: GO:0005829
    label: cytosol
  evidence_type: TAS
  original_reference_id: Reactome:R-HSA-9678749
  qualifier: located_in
  review:
    summary: >-
      Cytosolic localization from the Reactome "IMPDH tetramers bind IMPDH inhibitors"
      reaction (drug-binding context).
    action: ACCEPT
    reason: >-
      The cytosol is the correct core compartment for IMPDH1; the annotation arises
      from a drug/inhibitor-binding reaction but the localization itself is accurate
      and duplicates the other cytosol annotations.
    supported_by:
    - reference_id: file:human/IMPDH1/IMPDH1-uniprot.txt
      supporting_text: "SUBCELLULAR LOCATION: Cytoplasm"
- term:
    id: GO:0005829
    label: cytosol
  evidence_type: TAS
  original_reference_id: Reactome:R-HSA-9748945
  qualifier: located_in
  review:
    summary: >-
      Cytosolic localization from the Reactome "IMPDH tetramers dehydrogenate 6TIMP
      to 6TXMP" reaction (thiopurine metabolism context).
    action: ACCEPT
    reason: >-
      Correct core localization. The reaction reflects IMPDH turnover of a thiopurine
      metabolite, but the cytosolic assignment is accurate and duplicates the other
      cytosol annotations.
    supported_by:
    - reference_id: file:human/IMPDH1/IMPDH1-uniprot.txt
      supporting_text: "SUBCELLULAR LOCATION: Cytoplasm"
- term:
    id: GO:0003676
    label: nucleic acid binding
  evidence_type: IDA
  original_reference_id: PMID:14766016
  qualifier: enables
  review:
    summary: >-
      Direct demonstration that IMPDH (both human isoforms) binds single-stranded
      nucleic acids with nanomolar affinity via its CBS subdomain, with ~100
      nucleotides bound per tetramer.
    action: KEEP_AS_NON_CORE
    reason: >-
      This is a genuine, experimentally supported moonlighting activity of IMPDH1
      mediated by the regulatory CBS (Bateman) subdomain, and several RP10/LCA
      disease variants alter this nucleic-acid affinity. However, the enzyme's core
      function is IMP dehydrogenase catalysis; the nucleic-acid-binding role is
      regulatory/moonlighting and best retained as non-core.
    supported_by:
    - reference_id: PMID:14766016
      supporting_text: "bind single-stranded nucleic acids with nanomolar affinity via the"
- term:
    id: GO:0003677
    label: DNA binding
  evidence_type: IDA
  original_reference_id: PMID:14766016
  qualifier: enables
  review:
    summary: >-
      IMPDH was shown by immunoprecipitation to bind DNA (and RNA) in vivo, an
      extension of its single-stranded nucleic-acid-binding activity.
    action: KEEP_AS_NON_CORE
    reason: >-
      Experimentally supported (IDA) but represents the moonlighting nucleic-acid
      interaction rather than a sequence-specific transcription-factor-type DNA
      binding. It is a non-core, regulatory activity distinct from the enzyme's
      committed metabolic role; retained as non-core per curation policy for
      experimental annotations.
    supported_by:
    - reference_id: PMID:14766016
      supporting_text: "immunoprecipitation experiments show that IMPDH binds both RNA and DNA in vivo"
- term:
    id: GO:0005634
    label: nucleus
  evidence_type: IDA
  original_reference_id: PMID:14766016
  qualifier: located_in
  review:
    summary: >-
      Direct detection of IMPDH in the nucleus of human cells, consistent with its
      nucleic-acid-binding activity.
    action: KEEP_AS_NON_CORE
    reason: >-
      Experimentally supported nuclear pool, but the enzyme's core catalytic function
      in de novo GMP synthesis is cytosolic. Nuclear localization is tied to the
      moonlighting nucleic-acid-binding role and is non-core.
    supported_by:
    - reference_id: PMID:14766016
      supporting_text: "IMPDH is found in the nucleus of human cells, as"
- term:
    id: GO:0005737
    label: cytoplasm
  evidence_type: IDA
  original_reference_id: PMID:14766016
  qualifier: located_in
  review:
    summary: >-
      Direct localization of IMPDH to the cytoplasm, its principal compartment.
    action: ACCEPT
    reason: >-
      Experimental (IDA) cytoplasmic localization corroborates the core cytosolic
      residence where IMPDH1 catalyses IMP -> XMP; consistent with the IDA cytosol
      and IBA/IEA cytoplasm annotations.
    supported_by:
    - reference_id: file:human/IMPDH1/IMPDH1-uniprot.txt
      supporting_text: "SUBCELLULAR LOCATION: Cytoplasm"
- term:
    id: GO:0003938
    label: IMP dehydrogenase activity
  evidence_type: TAS
  original_reference_id: PMID:1969416
  qualifier: enables
  review:
    summary: >-
      Traceable author statement (original cloning paper) that the two human IMPDH
      cDNAs encode IMP dehydrogenase, the rate-limiting enzyme of de novo guanine
      nucleotide synthesis.
    action: ACCEPT
    reason: >-
      Core molecular function supported by the foundational cloning/characterization
      paper for human IMPDH1 (type I). Duplicates GO:0003938 by other evidence codes,
      which is acceptable.
    supported_by:
    - reference_id: PMID:1969416
      supporting_text: "IMP dehydrogenase (EC 1.1.1.205), the rate-limiting enzyme of de novo GTP"
core_functions:
- description: >-
    NAD+-dependent oxidation of inosine 5'-monophosphate (IMP) to xanthosine
    5'-monophosphate (XMP), the committed and rate-limiting step of de novo
    guanine-nucleotide biosynthesis, performed in the cytosol.
  molecular_function:
    id: GO:0003938
    label: IMP dehydrogenase activity
  directly_involved_in:
  - id: GO:0097294
    label: "'de novo' XMP biosynthetic process"
  locations:
  - id: GO:0005829
    label: cytosol
  supported_by:
  - reference_id: file:human/IMPDH1/IMPDH1-uniprot.txt
    supporting_text: "Reaction=IMP + NAD(+) + H2O = XMP + NADH + H(+)"
  - reference_id: PMID:1969416
    supporting_text: "IMP dehydrogenase (EC 1.1.1.205), the rate-limiting enzyme of de novo GTP"
- description: >-
    Committed step of de novo GMP biosynthesis: by producing XMP, IMPDH1 supplies
    the immediate precursor that GMP synthase aminates to GMP, thereby providing
    guanine nucleotides for the cell.
  molecular_function:
    id: GO:0003938
    label: IMP dehydrogenase activity
  directly_involved_in:
  - id: GO:0006177
    label: GMP biosynthetic process
  locations:
  - id: GO:0005829
    label: cytosol
  supported_by:
  - reference_id: file:human/IMPDH1/IMPDH1-uniprot.txt
    supporting_text: "step in the de novo synthesis of guanine nucleotides"
proposed_new_terms: []
suggested_questions:
- question: >-
    Is the single-stranded nucleic-acid-binding activity of the CBS subdomain a
    physiologically regulated function of IMPDH1 in photoreceptors, and does its
    disruption (as by RP10/LCA CBS-domain variants) cause disease independently of
    catalytic activity?
- question: >-
    What is the functional significance of IMPDH1 filament ("rods and rings"/
    cytoophidia) assembly for guanine-nucleotide homeostasis in the retina?
suggested_experiments:
- description: >-
    Separate-of-function IMPDH1 alleles that abolish catalysis while preserving
    nucleic-acid binding (and vice versa) expressed in photoreceptor models, to test
    whether RP10/LCA phenotypes arise from loss of catalysis, loss/alteration of
    nucleic-acid binding, or a dominant-negative filament defect.
- description: >-
    Cryo-EM and cellular imaging of retina-specific IMPDH1 isoforms to determine how
    the extended N-terminal retinal isoforms modulate filament assembly and
    GTP/GDP-dependent allosteric regulation.