MOCS3

UniProt ID: O95396
Organism: Homo sapiens
Review Status: IN PROGRESS
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Gene Description

MOCS3 (also called UBA4) is a cytosolic, two-domain bifunctional enzyme that activates the C-terminal Gly-Gly motif of small Ξ²-grasp-fold sulfur-carrier proteins for two sulfur-relay pathways. Its N-terminal HesA/MoeB/ThiF (E1-like) adenylation domain forms an acyl-adenylate (-COAMP) at the C-terminus of its substrates, and its C-terminal rhodanese-like domain then transfers persulfide sulfur from a catalytic cysteine (Cys412) to convert this into a C-terminal thiocarboxylate (-COSH). Through these coupled adenylyltransferase (EC 2.7.7.80) and sulfurtransferase (EC 2.8.1.11) activities, MOCS3 thiocarboxylates MOCS2A, the small subunit of molybdopterin (MPT) synthase, driving molybdenum cofactor (Moco) biosynthesis, and it thiocarboxylates the ubiquitin-related modifier URM1, supplying the sulfur used for 2-thiolation of wobble uridine (mcm5s2U34) in cytosolic tRNA-Lys, tRNA-Glu and tRNA-Gln. The physiological sulfur donor to the MOCS3 rhodanese domain is the cytosolic L-cysteine desulfurase NFS1 rather than thiosulfate. MOCS3 binds one zinc ion per subunit and requires ATP. Loss-of-function variants cause molybdenum cofactor deficiency type B2.

Existing Annotations Review

GO Term Evidence Action Reason
GO:0005737 cytoplasm
IBA
GO_REF:0000033
ACCEPT
Summary: MOCS3 acts in the cytoplasm/cytosol. Location is well supported by direct biochemical fractionation and imaging, though cytosol (GO:0005829) is the more precise term and is separately annotated by IDA.
Supporting Evidence:
file:human/MOCS3/MOCS3-uniprot.txt
Cytoplasm, cytosol
GO:0016779 nucleotidyltransferase activity
IBA
GO_REF:0000033
KEEP AS NON CORE
Summary: Correct but general. MOCS3 transfers AMP onto the C-terminal Gly-Gly of its sulfur-carrier substrates; the specific child term molybdopterin-synthase adenylyltransferase activity (GO:0061605) is the informative core MF.
Supporting Evidence:
file:human/MOCS3/MOCS3-uniprot.txt
Its N-terminus first activates URM1 and MOCS2A as acyl-adenylates (-COAMP)
GO:0004792 thiosulfate-cyanide sulfurtransferase activity
IBA
GO_REF:0000033
MARK AS OVER ANNOTATED
Summary: The isolated MOCS3 rhodanese-like domain can transfer sulfur from thiosulfate to cyanide in vitro, but at ~1000-fold lower activity than bovine rhodanese, and thiosulfate is explicitly not the physiological sulfur donor in humans; NFS1 is. This is an in-vitro rhodanese activity, not the biological function.
Propagation Review
Root cause: TERM SCOPING PROBLEM
Failure modes: FUNCTIONAL DIVERGENCE
Sources checked:
PANTHER:PTN000102040 Β· MOCS3/UBA4 family node SUPPORTS SOURCE BUT NOT TARGET
Supporting Evidence:
PMID:17459099
in humans and most eukaryotes thiosulfate is not the physiologic sulfur donor for MOCS3
GO:0042292 URM1 activating enzyme activity
IBA
GO_REF:0000033
ACCEPT
Summary: MOCS3 activates the ubiquitin-related modifier URM1 (adenylation followed by thiocarboxylation of its C-terminal glycine), the URM1-pathway counterpart of its MOCS2A activity. This is a core molecular function, also supported by IDA (PMID:19017811).
Supporting Evidence:
file:human/MOCS3/MOCS3-uniprot.txt
Acts by mediating the C-terminal thiocarboxylation of sulfur carriers URM1 and MOCS2A
GO:0002143 tRNA wobble position uridine thiolation
IBA
GO_REF:0000033
ACCEPT
Summary: MOCS3 is required for 2-thiolation of wobble uridine (mcm5s2U34) of cytosolic tRNA-Lys, tRNA-Glu and tRNA-Gln, by activating URM1 as the sulfur carrier. Core biological process, also supported by IDA and IMP.
Supporting Evidence:
file:human/MOCS3/MOCS3-uniprot.txt
Plays a central role in 2-thiolation of mcm(5)S(2)U at tRNA wobble positions of cytosolic tRNA(Lys), tRNA(Glu) and tRNA(Gln)
GO:0032447 protein urmylation
IBA
GO_REF:0000033
KEEP AS NON CORE
Summary: MOCS3 is a component of the URM1 machinery (it activates URM1), and URM1 can be conjugated to lysines of target proteins including MOCS3 itself under oxidative stress. This is a downstream/secondary role rather than the core catalytic function, which is sulfur-carrier activation.
Supporting Evidence:
PMID:21209336
Urm1 is appended to lysine residues of three components that function in its own pathway (i.e., MOCS3, ATPBD3, and CTU2)
GO:0002098 tRNA wobble uridine modification
IEA
GO_REF:0000104
KEEP AS NON CORE
Summary: Correct but a broader parent of tRNA wobble position uridine thiolation (GO:0002143). MOCS3 specifically contributes the 2-thiolation (thio) step, so the more specific thiolation term is preferred as core.
Supporting Evidence:
file:human/MOCS3/MOCS3-uniprot.txt
Plays a central role in 2-thiolation of mcm(5)S(2)U at tRNA wobble positions of cytosolic tRNA(Lys), tRNA(Glu) and tRNA(Gln)
GO:0002143 tRNA wobble position uridine thiolation
IEA
GO_REF:0000002
ACCEPT
Summary: Same core biological process as the IBA/IDA/IMP annotations to this term; electronic support from InterPro is consistent with the experimental evidence.
Supporting Evidence:
file:human/MOCS3/MOCS3-uniprot.txt
Plays a central role in 2-thiolation of mcm(5)S(2)U at tRNA wobble positions of cytosolic tRNA(Lys), tRNA(Glu) and tRNA(Gln)
GO:0005737 cytoplasm
IEA
GO_REF:0000104
ACCEPT
Summary: Consistent with the experimentally established cytosolic localization; cytosol (GO:0005829) is the more precise term also annotated by IDA.
Supporting Evidence:
file:human/MOCS3/MOCS3-uniprot.txt
Cytoplasm, cytosol
GO:0005829 cytosol
IEA
GO_REF:0000120
ACCEPT
Summary: Cytosol is the experimentally verified location of MOCS3 (subcellular fractionation and confocal imaging in HeLa cells). This is the precise CC term.
Supporting Evidence:
file:human/MOCS3/MOCS3-uniprot.txt
Cytoplasm, cytosol
GO:0006777 Mo-molybdopterin cofactor biosynthetic process
IEA
GO_REF:0000104
ACCEPT
Summary: MOCS3 is essential for molybdenum cofactor biosynthesis; it thiocarboxylates MOCS2A, the small MPT synthase subunit. Core BP, extensively supported by IDA/IMP.
Supporting Evidence:
file:human/MOCS3/MOCS3-uniprot.txt
Also essential during biosynthesis of the molybdenum cofactor
GO:0008641 ubiquitin-like modifier activating enzyme activity
IEA
GO_REF:0000002
MARK AS OVER ANNOTATED
Summary: Family-level (E1-like/MoeB) inference. MOCS3 does activate the ubiquitin-related modifier URM1, but as a sulfur carrier (adenylation then thiocarboxylation of the C-terminal glycine) rather than by a classic Ubl conjugation cascade; no thioester intermediate is formed with URM1/MOCS2A. The specific term URM1 activating enzyme activity (GO:0042292) is preferred.
Proposed replacements: URM1 activating enzyme activity
Supporting Evidence:
PMID:18491921
The formation of a thioester intermediate between Uba4 and Urm1 or MOCS2A was not observed
GO:0016779 nucleotidyltransferase activity
IEA
GO_REF:0000104
KEEP AS NON CORE
Summary: Correct but general parent of molybdopterin-synthase adenylyltransferase activity (GO:0061605). The specific child is the informative core MF.
Supporting Evidence:
file:human/MOCS3/MOCS3-uniprot.txt
Its N-terminus first activates URM1 and MOCS2A as acyl-adenylates (-COAMP)
GO:0016783 sulfurtransferase activity
IEA
GO_REF:0000120
KEEP AS NON CORE
Summary: Correct but general parent of the specific molybdopterin-synthase sulfurtransferase activity (GO:0061604). MOCS3 forms a persulfide on Cys412 and transfers sulfur to its carrier-protein substrate.
Supporting Evidence:
file:human/MOCS3/MOCS3-uniprot.txt
the persulfide sulfur on the catalytic cysteine is transferred to URM1 and MOCS2A to form thiocarboxylation
GO:0034227 tRNA thio-modification
IEA
GO_REF:0000104
ACCEPT
Summary: MOCS3 supplies the sulfur (via URM1) for thio-modification of cytosolic tRNA. Core BP, also supported by IDA/IMP.
Supporting Evidence:
file:human/MOCS3/MOCS3-uniprot.txt
Plays a central role in 2-thiolation of mcm(5)S(2)U at tRNA wobble positions
GO:0061604 molybdopterin-synthase sulfurtransferase activity
IEA
GO_REF:0000120
ACCEPT
Summary: Specific core catalytic MF (EC 2.8.1.11): transfer of persulfide sulfur from the rhodanese-like domain onto the adenylylated MOCS2A C-terminus to form a thiocarboxylate. Strongly supported by EXP/IDA/IMP.
Supporting Evidence:
file:human/MOCS3/MOCS3-uniprot.txt
EC=2.8.1.11
GO:0061605 molybdopterin-synthase adenylyltransferase activity
IEA
GO_REF:0000120
ACCEPT
Summary: Specific core catalytic MF (EC 2.7.7.80): ATP-dependent adenylation of the MOCS2A C-terminal Gly-Gly. Strongly supported by EXP/IDA/ISO.
Supporting Evidence:
file:human/MOCS3/MOCS3-uniprot.txt
EC=2.7.7.80
GO:0070566 adenylyltransferase activity
IEA
GO_REF:0000002
KEEP AS NON CORE
Summary: Correct but general parent of molybdopterin-synthase adenylyltransferase activity (GO:0061605). The specific child is the informative core MF.
Supporting Evidence:
file:human/MOCS3/MOCS3-uniprot.txt
Its N-terminus first activates URM1 and MOCS2A as acyl-adenylates (-COAMP)
GO:0005515 protein binding
IPI
PMID:21209336
Role of the ubiquitin-like protein Urm1 as a noncanonical ly...
MARK AS OVER ANNOTATED
Summary: IntAct-curated interaction with URM1 (Q9BTM9), the sulfur-carrier substrate of MOCS3. Bare "protein binding" is uninformative; the functionally meaningful relationship is captured by URM1 activating enzyme activity (GO:0042292) and the adenylyl/sulfurtransferase MF terms.
GO:0005515 protein binding
IPI
PMID:32814053
Interactome Mapping Provides a Network of Neurodegenerative ...
MARK AS OVER ANNOTATED
Summary: Large-scale neurodegenerative-disease interactome (yeast two-hybrid) reporting candidate interactions of MOCS3 with ATXN1 (P54253) and TARDBP/TDP-43 (Q13148). Bare "protein binding" is uninformative and these high-throughput interactions have no dedicated functional follow-up for MOCS3.
GO:0061604 molybdopterin-synthase sulfurtransferase activity
EXP
PMID:18650437
A novel role for human Nfs1 in the cytoplasm: Nfs1 acts as a...
ACCEPT
Summary: Experimental support: the sulfurated MOCS3 rhodanese-like domain, receiving sulfur from NFS1, thiocarboxylates MOCS2A. Core sulfurtransferase MF.
Supporting Evidence:
PMID:18650437
The two-domain protein MOCS3 catalyzes both the adenylation and the subsequent generation of a thiocarboxylate group at the C terminus of MOCS2A by its C-terminal rhodanese-like domain (RLD)
GO:0061604 molybdopterin-synthase sulfurtransferase activity
EXP
PMID:22453920
Dual role of the molybdenum cofactor biosynthesis protein MO...
ACCEPT
Summary: Experimental support: MOCS3 forms the thiocarboxylate on the MOCS2A C-terminus via a sulfur-transfer step following adenylation. Core sulfurtransferase MF.
Supporting Evidence:
PMID:22453920
MOCS3 activates both MOCS2A and URM1 by adenylation and a subsequent sulfur transfer step for the formation of the thiocarboxylate group at the C terminus of each protein
GO:0061605 molybdopterin-synthase adenylyltransferase activity
EXP
PMID:18650437
A novel role for human Nfs1 in the cytoplasm: Nfs1 acts as a...
ACCEPT
Summary: Experimental support: the two-domain MOCS3 catalyzes the ATP-dependent adenylation of the MOCS2A C-terminus. Core adenylyltransferase MF.
Supporting Evidence:
PMID:18650437
The two-domain protein MOCS3 catalyzes both the adenylation and the subsequent generation of a thiocarboxylate group
GO:0002143 tRNA wobble position uridine thiolation
IDA
PMID:22453920
Dual role of the molybdenum cofactor biosynthesis protein MO...
ACCEPT
Summary: Direct evidence that MOCS3 functions in the tRNA-thiolation branch (via URM1) in human cells. Core BP.
Supporting Evidence:
PMID:22453920
We studied two pathways that involve the transfer of persulfide sulfur in humans, molybdenum cofactor biosynthesis and tRNA thiolation
GO:0005829 cytosol
IDA
PMID:22453920
Dual role of the molybdenum cofactor biosynthesis protein MO...
ACCEPT
Summary: MOCS3 is localized to the cytosol; C-terminal extension of its substrates relocalizes MOCS3 from cytosol to nucleus, confirming cytosolic steady-state localization. Core CC.
Supporting Evidence:
PMID:22453920
altered the localization of MOCS3 from the cytosol to the nucleus
GO:0006777 Mo-molybdopterin cofactor biosynthetic process
IDA
PMID:22453920
Dual role of the molybdenum cofactor biosynthesis protein MO...
ACCEPT
Summary: Direct evidence that MOCS3 functions in molybdenum cofactor biosynthesis in human cells. Core BP.
Supporting Evidence:
PMID:22453920
molybdenum cofactor biosynthesis and tRNA thiolation
GO:0061605 molybdopterin-synthase adenylyltransferase activity
IDA
PMID:22453920
Dual role of the molybdenum cofactor biosynthesis protein MO...
ACCEPT
Summary: Direct experimental support for the adenylyltransferase activity on MOCS2A (and URM1). Core MF.
Supporting Evidence:
PMID:22453920
MOCS3 activates both MOCS2A and URM1 by adenylation
GO:0070566 adenylyltransferase activity
IDA
PMID:22453920
Dual role of the molybdenum cofactor biosynthesis protein MO...
KEEP AS NON CORE
Summary: Correct but general parent of molybdopterin-synthase adenylyltransferase activity (GO:0061605); the specific child is the core MF.
Supporting Evidence:
PMID:22453920
MOCS3 activates both MOCS2A and URM1 by adenylation
GO:0005829 cytosol
IDA
PMID:23593335
The L-cysteine desulfurase NFS1 is localized in the cytosol ...
ACCEPT
Summary: Subcellular fractionation and confocal imaging place MOCS3 (and its interaction with NFS1) in the cytosol of HeLa cells. Core CC.
Supporting Evidence:
PMID:23593335
these results show that NFS1 interacts with MOCS3 in the cytosol
GO:0006777 Mo-molybdopterin cofactor biosynthetic process
IDA
PMID:18650437
A novel role for human Nfs1 in the cytoplasm: Nfs1 acts as a...
ACCEPT
Summary: Direct evidence for MOCS3's role in Moco biosynthesis, with NFS1 supplying the sulfur to the MOCS3 rhodanese domain in the cytosol. Core BP.
Supporting Evidence:
PMID:18650437
cytosolic Nfs1 has an important role in sulfur transfer for the biosynthesis of Moco
GO:0061605 molybdopterin-synthase adenylyltransferase activity
ISO
PMID:18491921
The sulfurtransferase activity of Uba4 presents a link betwe...
ACCEPT
Summary: Orthology-based support from yeast Uba4 (UniProtKB:P38820), whose N-terminal domain adenylates MOCS2A/Urm1 as an acyl-adenylate; consistent with the human EXP/IDA evidence. Core MF.
Supporting Evidence:
PMID:18491921
The N-terminal domain of Uba4 catalyzes the activation of either MOCS2A or Urm1 by formation of an acyl-adenylate bond
GO:0061604 molybdopterin-synthase sulfurtransferase activity
IMP
PMID:15073332
Evidence for the physiological role of a rhodanese-like prot...
ACCEPT
Summary: Mutation of the persulfide-forming active-site cysteine (Cys412) abolishes MOCS3-RLD sulfurtransferase activity, and the sulfurated RLD provides sulfur for MOCS2A thiocarboxylation. Core MF.
Supporting Evidence:
PMID:15073332
Mutation of the putative persulfide-forming active-site cysteine residue C412 abolished the sulfurtransferase activity of MOCS3-RLD completely
GO:0016783 sulfurtransferase activity
IMP
PMID:17459099
Site-directed mutagenesis of the active site loop of the rho...
KEEP AS NON CORE
Summary: Active-site loop mutagenesis of the MOCS3 rhodanese domain characterizes its sulfurtransferase activity, but this general term is superseded by the specific molybdopterin-synthase sulfurtransferase activity (GO:0061604).
Supporting Evidence:
PMID:17459099
the charge of the last amino acid determines thiosulfate sulfurtransferase activity
GO:0061604 molybdopterin-synthase sulfurtransferase activity
IDA
PMID:15073332
Evidence for the physiological role of a rhodanese-like prot...
ACCEPT
Summary: Direct in-vitro demonstration that sulfurated MOCS3-RLD provides sulfur for MOCS2A thiocarboxylation. Core MF.
Supporting Evidence:
PMID:15073332
the sulfurated form of MOCS3-RLD was able to provide the sulfur for the thiocarboxylation of MOCS2A
GO:0005515 protein binding
IPI
PMID:23593335
The L-cysteine desulfurase NFS1 is localized in the cytosol ...
MARK AS OVER ANNOTATED
Summary: IntAct/UniProt-curated interaction with NFS1 (Q9Y697), the cytosolic sulfur donor, via the MOCS3 rhodanese-like domain. Bare "protein binding" is uninformative; the functional relationship (NFS1 as sulfur donor) is captured in the sulfurtransferase MF and Moco/tRNA BP terms.
GO:0006777 Mo-molybdopterin cofactor biosynthetic process
IMP
PMID:30817134
Analysis of the Cellular Roles of MOCS3 Identifies a MOCS3-I...
ACCEPT
Summary: CRISPR MOCS3 knockout in HEK293T cells abolishes sulfite oxidase activity due to absence of Moco, demonstrating an essential in-vivo role in Moco biosynthesis. Core BP.
Supporting Evidence:
PMID:30817134
sulfite oxidase activity was almost completely abolished, on the basis of the absence of Moco in these cells
GO:0034227 tRNA thio-modification
IMP
PMID:30817134
Analysis of the Cellular Roles of MOCS3 Identifies a MOCS3-I...
ACCEPT
Summary: In the MOCS3 knockout, mcm5s2U thio-modified tRNAs are undetectable, showing MOCS3 is required for cytosolic tRNA thio-modification. Core BP.
Supporting Evidence:
PMID:30817134
mcm5s2U thio-modified tRNAs were not detectable
GO:0005829 cytosol
TAS
Reactome:R-HSA-947514
ACCEPT
Summary: Reactome traceable-author statement for the cytosolic NFS1-to-MOCS3 sulfur transfer reaction. Consistent with the experimentally established cytosolic localization.
Supporting Evidence:
file:human/MOCS3/MOCS3-uniprot.txt
Cytoplasm, cytosol
GO:0005829 cytosol
TAS
Reactome:R-HSA-947538
ACCEPT
Summary: Reactome traceable-author statement for the cytosolic MOCS3-to-MOCS2A sulfur transfer reaction. Consistent with the experimentally established cytosolic localization.
Supporting Evidence:
file:human/MOCS3/MOCS3-uniprot.txt
Cytoplasm, cytosol
GO:0016783 sulfurtransferase activity
IDA
PMID:18650437
A novel role for human Nfs1 in the cytoplasm: Nfs1 acts as a...
KEEP AS NON CORE
Summary: Direct evidence for the rhodanese-domain sulfurtransferase activity; superseded by the specific molybdopterin-synthase sulfurtransferase activity (GO:0061604) as core.
Supporting Evidence:
PMID:18650437
the subsequent generation of a thiocarboxylate group at the C terminus of MOCS2A by its C-terminal rhodanese-like domain (RLD)
GO:0002098 tRNA wobble uridine modification
IDA
PMID:19017811
A functional proteomics approach links the ubiquitin-related...
KEEP AS NON CORE
Summary: Direct evidence linking the URM1 pathway (activated by MOCS3) to tRNA modification. Broader parent of tRNA wobble position uridine thiolation (GO:0002143), which is the more specific core term.
Supporting Evidence:
PMID:19017811
an enzymatic activity that links Urm1 to a tRNA modification pathway
GO:0005515 protein binding
IPI
PMID:18650437
A novel role for human Nfs1 in the cytoplasm: Nfs1 acts as a...
MARK AS OVER ANNOTATED
Summary: IntAct/UniProt-curated interaction with NFS1 (Q9Y697), the sulfur donor that interacts specifically with the MOCS3 rhodanese-like domain. Bare "protein binding" is uninformative; the specific MF/BP terms capture the biology.
GO:0005829 cytosol
IDA
PMID:15073332
Evidence for the physiological role of a rhodanese-like prot...
ACCEPT
Summary: MOCS3 and the MPT synthase subunits are localized in the cytosol, unlike most mammalian rhodaneses which are mitochondrial. Core CC.
Supporting Evidence:
PMID:15073332
MOCS3 in addition to the subunits of MPT synthase are localized in the cytosol
GO:0006777 Mo-molybdopterin cofactor biosynthetic process
IDA
PMID:15073332
Evidence for the physiological role of a rhodanese-like prot...
ACCEPT
Summary: Establishes the physiological role of MOCS3 in human molybdenum cofactor biosynthesis. Core BP.
Supporting Evidence:
PMID:15073332
the physiological role of a rhodanese-like protein for the biosynthesis of the molybdenum cofactor in humans
GO:0016779 nucleotidyltransferase activity
IDA
PMID:19017811
A functional proteomics approach links the ubiquitin-related...
KEEP AS NON CORE
Summary: Correct but general parent of molybdopterin-synthase adenylyltransferase activity (GO:0061605); the specific child is the core MF.
Supporting Evidence:
PMID:19017811
Urm1 is activated by an unusual mechanism to yield a thiocarboxylate intermediate
GO:0016783 sulfurtransferase activity
IDA
PMID:19017811
A functional proteomics approach links the ubiquitin-related...
KEEP AS NON CORE
Summary: Direct evidence for the sulfur-transfer step generating the URM1 thiocarboxylate; superseded by the specific molybdopterin-synthase sulfurtransferase activity (GO:0061604) as core.
Supporting Evidence:
PMID:19017811
Urm1 is activated by an unusual mechanism to yield a thiocarboxylate intermediate that serves as sulfur donor in tRNA thiolation reactions
GO:0034227 tRNA thio-modification
IDA
PMID:19017811
A functional proteomics approach links the ubiquitin-related...
ACCEPT
Summary: Direct evidence that the URM1 thiocarboxylate (formed by MOCS3) serves as sulfur donor for tRNA thiolation. Core BP.
Supporting Evidence:
PMID:19017811
a thiocarboxylate intermediate that serves as sulfur donor in tRNA thiolation reactions
GO:0042292 URM1 activating enzyme activity
IDA
PMID:19017811
A functional proteomics approach links the ubiquitin-related...
ACCEPT
Summary: Direct evidence that MOCS3 activates URM1 to its thiocarboxylate form. Core MF for the tRNA-thiolation branch.
Supporting Evidence:
PMID:19017811
Urm1 is activated by an unusual mechanism to yield a thiocarboxylate intermediate
GO:0016783 sulfurtransferase activity
IDA
PMID:15910006
Molybdenum cofactor biosynthesis in humans: identification o...
KEEP AS NON CORE
Summary: Mass spectrometry directly identifies the catalytic persulfide on Cys412 of the MOCS3 rhodanese domain, the chemical basis of its sulfurtransferase activity; superseded by the specific term GO:0061604 as core.
Supporting Evidence:
PMID:15910006
direct evidence for the formation of a persulfide group that is exclusively formed on C412

Core Functions

ATP-dependent adenylyltransferase that activates the C-terminal Gly-Gly of the molybdopterin-synthase small subunit MOCS2A (and of URM1) by forming an acyl-adenylate, the first step of sulfur-carrier activation.

Supporting Evidence:
  • PMID:22453920
    MOCS3 activates both MOCS2A and URM1 by adenylation and a subsequent sulfur transfer step for the formation of the thiocarboxylate group at the C terminus of each protein
  • file:human/MOCS3/MOCS3-uniprot.txt
    EC=2.7.7.80

Rhodanese-like-domain sulfurtransferase that transfers persulfide sulfur from its catalytic Cys412 onto the adenylylated C-terminus of MOCS2A to form a thiocarboxylate, using NFS1 as the physiological sulfur donor; drives molybdenum cofactor biosynthesis.

Supporting Evidence:
  • PMID:15073332
    the sulfurated form of MOCS3-RLD was able to provide the sulfur for the thiocarboxylation of MOCS2A
  • PMID:15073332
    Mutation of the putative persulfide-forming active-site cysteine residue C412 abolished the sulfurtransferase activity of MOCS3-RLD completely

Activates the ubiquitin-related modifier URM1 by adenylation and subsequent thiocarboxylation of its C-terminal glycine, generating the sulfur-carrier thiocarboxylate used for 2-thiolation of wobble uridine (mcm5s2U34) in cytosolic tRNA-Lys, tRNA-Glu and tRNA-Gln.

Supporting Evidence:
  • PMID:19017811
    Urm1 is activated by an unusual mechanism to yield a thiocarboxylate intermediate that serves as sulfur donor in tRNA thiolation reactions
  • PMID:30817134
    mcm5s2U thio-modified tRNAs were not detectable

References

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Suggested Questions for Experts

Q: What reductant regenerates oxidized MOCS3 (resolving the transient Cys316-Cys324 disulfide) between catalytic cycles in vivo? Reactome notes this reducing agent is unknown.

Q: Are the Moco-biosynthesis and tRNA-thiolation branches of MOCS3 differentially regulated or competitively partitioned in cells, and does substrate (MOCS2A vs URM1) availability control flux?

Suggested Experiments

Experiment: Quantitatively partition MOCS3 activity between the MOCS2A (Moco) and URM1 (tRNA thiolation) branches in the MOCS3-knockout HEK293T background by add-back of catalytic (Cys412) and adenylation (Cys239/ACT_SITE) point mutants, measuring both sulfite oxidase activity and mcm5s2U tRNA levels.

Experiment: Test the physiological relevance and functional consequence of the MOCS3-ATXN1/TARDBP high-throughput interactions with orthogonal co-IP/proximity assays in relevant cell types, to determine whether they reflect genuine function or interactome noise.

πŸ“š Additional Documentation

Notes

(MOCS3-notes.md)

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