MTX1

UniProt ID: Q13505
Organism: Homo sapiens
Review Status: COMPLETE
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Gene Description

MTX1 (metaxin-1) is a non-enzymatic mitochondrial outer membrane metaxin-family accessory subunit of the mammalian SAM complex. Its best-supported core role is structural support of SAM-mediated insertion and assembly of beta-barrel outer membrane proteins such as VDACs and TOM40, with additional non-core links to MIB/MICOS-associated organization and mitochondrial quality control.

Existing Annotations Review

GO Term Evidence Action Reason
GO:0045040 protein insertion into mitochondrial outer membrane
IBA
GO_REF:0000033
ACCEPT
Summary: Correct phylogenetic inference. MTX1 is an accessory SAM subunit involved in SAM-mediated insertion/assembly of beta-barrel proteins into the mitochondrial outer membrane.
Supporting Evidence:
file:human/MTX1/MTX1-deep-research-falcon.md
MTX1 is best supported as an **accessory subunit of the mitochondrial Sorting and Assembly Machinery (SAM)** required for efficient **import/assembly of Ξ²-barrel proteins** into the OMM
GO:0070096 mitochondrial outer membrane translocase complex assembly
IBA
GO_REF:0000033
ACCEPT
Summary: Accepted as a SAM-related assembly role. MTX1 supports assembly of beta-barrel outer membrane proteins, including TOM complex components such as TOM40, rather than acting as a catalytic enzyme.
GO:0001401 SAM complex
IBA
GO_REF:0000033
ACCEPT
Summary: Correct. MTX1 is a metaxin-family accessory subunit of the mammalian SAM complex with SAMM50, MTX2, and MTX3.
GO:0001401 SAM complex
IEA
GO_REF:0000002
ACCEPT
Summary: Correct InterPro-derived complex annotation. The Falcon review identifies MTX1 as a mammalian SAM complex accessory subunit.
GO:0005741 mitochondrial outer membrane
IEA
GO_REF:0000044
ACCEPT
Summary: Correct UniProt-derived localization. MTX1 is an outer mitochondrial membrane SAM/metaxin protein.
GO:0016020 membrane
IEA
GO_REF:0000044
MARK AS OVER ANNOTATED
Summary: Correct but overly general. MTX1 is specifically localized to the mitochondrial outer membrane and SAM complex.
Reason: Subsumed by mitochondrial outer membrane and SAM complex annotations.
GO:0007595 lactation
IEA
GO_REF:0000107
REMOVE
Summary: This automatic orthology transfer does not match the synthesized function of human MTX1 as a mitochondrial SAM accessory factor. Falcon research did not identify MTX1-specific evidence for lactation as a gene-level core process.
Reason: Unsupported organism-level phenotype transfer; not part of the curated MTX1 mitochondrial SAM function.
GO:0001401 SAM complex
IPI
PMID:17510655
Conserved roles of Sam50 and metaxins in VDAC biogenesis.
ACCEPT
Summary: Accepted. Conserved roles of metaxins and Sam50 in VDAC biogenesis support MTX1 membership in the SAM complex.
GO:0005741 mitochondrial outer membrane
NAS
PMID:31387448
Mitochondria-hubs for regulating cellular biochemistry: emer...
ACCEPT
Summary: Accepted. MTX1 is consistently described as a mitochondrial outer membrane metaxin/SAM component.
GO:0045040 protein insertion into mitochondrial outer membrane
NAS
PMID:31387448
Mitochondria-hubs for regulating cellular biochemistry: emer...
ACCEPT
Summary: Accepted as the core MTX1 process. MTX1 acts as a non-catalytic accessory factor for SAM-mediated insertion/assembly of beta-barrel proteins into the mitochondrial outer membrane.
GO:0005739 mitochondrion
HTP
PMID:34800366
Quantitative high-confidence human mitochondrial proteome an...
MARK AS OVER ANNOTATED
Summary: Correct but too general. MTX1 is specifically localized to the mitochondrial outer membrane and SAM complex.
Reason: Subsumed by mitochondrial outer membrane and SAM complex annotations.
GO:0005515 protein binding
IPI
PMID:31644573
Armadillo repeat-containing protein 1 is a dual localization...
REMOVE
Summary: Protein binding is too generic to represent MTX1 function. The relevant biology is membership in SAM and non-core association with MIB/MICOS-linked assemblies.
Reason: Generic protein binding is uninformative; complex membership and mitochondrial assembly terms capture the supported function.
GO:0001401 SAM complex
HDA
PMID:26477565
Evolution and structural organization of the mitochondrial c...
ACCEPT
Summary: Accepted. Affinity/proteomics evidence supports MTX1 in SAM-containing mitochondrial outer membrane assemblies.
GO:0007007 inner mitochondrial membrane organization
IC
PMID:26477565
Evolution and structural organization of the mitochondrial c...
KEEP AS NON CORE
Summary: Plausible secondary consequence of MTX1 association with MIB/MICOS-linked assemblies, but not the primary curated function compared with SAM-mediated outer membrane beta-barrel biogenesis.
Reason: MIB/MICOS-associated architecture is supported as an additional role, not the core MTX1 function.
GO:0140275 MIB complex
HDA
PMID:26477565
Evolution and structural organization of the mitochondrial c...
KEEP AS NON CORE
Summary: Supported as an additional MTX1-associated complex context. Keep as non-core because the strongest synthesized function is SAM-mediated beta-barrel outer membrane protein biogenesis.
Reason: MIB/MICOS association is secondary to the core SAM complex role.
GO:0016020 membrane
TAS
PMID:8660965
Structure and organization of the human metaxin gene (MTX) a...
MARK AS OVER ANNOTATED
Summary: Correct but too general. MTX1 is specifically a mitochondrial outer membrane metaxin/SAM protein.
Reason: Subsumed by mitochondrial outer membrane and SAM complex annotations.

Core Functions

MTX1 is a structural/accessory metaxin subunit of the mammalian SAM complex that supports insertion and assembly of beta-barrel proteins into the mitochondrial outer membrane.

Supporting Evidence:
  • file:human/MTX1/MTX1-deep-research-falcon.md
    MTX1 is best supported as an **accessory subunit of the mitochondrial Sorting and Assembly Machinery (SAM)** required for efficient **import/assembly of Ξ²-barrel proteins** into the OMM
  • file:human/MTX1/MTX1-deep-research-falcon.md
    Mammalian SAM contains **SAMM50/SAM50** plus **MTX1, MTX2, MTX3**
  • file:human/MTX1/MTX1-deep-research-falcon.md
    MTX1 is an **outer mitochondrial membrane (OMM)** protein

References

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Suggested Questions for Experts

Q: Which human MTX1 surfaces are required for SAM-mediated VDAC/TOM40 beta-barrel assembly versus MIB/MICOS-associated interactions?

Q: Is MTX1 turnover by LC3C/p62-linked piecemeal mitophagy regulated by the same interfaces used for SAM complex assembly?

Suggested Experiments

Experiment: Use endogenous MTX1 knockout/rescue with domain or interface mutants, then assay SAM complex assembly, VDAC1/TOM40 import and assembly, mitochondrial ultrastructure, and LC3C/p62-dependent MTX1 turnover.

Hypothesis: MTX1 contributes separable interfaces for SAM beta-barrel assembly and MIB/MICOS-associated mitochondrial architecture.

Type: endogenous rescue, import assay, and mitochondrial proteomics

Deep Research

Falcon

(MTX1-deep-research-falcon.md)

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