NDUFA11 (B14.7; CI-B14.7) is a small (141-residue) multi-pass membrane accessory ("supernumerary") subunit of mitochondrial respiratory chain Complex I (NADH:ubiquinone oxidoreductase). It is nuclear-encoded on chromosome 19, imported into mitochondria, and is a stable structural subunit of the membrane arm of the mature holoenzyme, embedded in the inner mitochondrial membrane with its bulk on the matrix side and two transmembrane helices. NDUFA11 adopts a Tim17/Tim22/Tim23-like fold but functions as a genuine structural component of Complex I rather than as a protein translocase or insertase. It is not one of the 14 conserved catalytic core subunits and carries no redox cofactor, so it is non-catalytic; its role is structural, contributing to the assembly and stability of the membrane arm. Complex I as a whole transfers electrons from NADH to ubiquinone coupled to proton translocation across the inner membrane, feeding the respiratory chain and oxidative phosphorylation. In humans, recessive loss-of-function variants in NDUFA11 cause mitochondrial complex I deficiency nuclear type 14 (MC1DN14).
| GO Term | Evidence | Action | Reason |
|---|---|---|---|
| GO:0045271 respiratory chain complex I | IBA GO_REF:0000033 | ACCEPT | Summary: Phylogenetic (IBA) inference that NDUFA11 is part of respiratory chain complex I. Correct and strongly supported by direct experimental evidence: NDUFA11/B14.7 is a bona fide stable subunit of the Complex I holoenzyme. Core cellular-component annotation. Supporting Evidence: file:human/NDUFA11/NDUFA11-uniprot.txt Complex I is composed of 45 different subunits. |
| GO:0005743 mitochondrial inner membrane | IEA GO_REF:0000120 | ACCEPT | Summary: Electronic annotation to mitochondrial inner membrane. Consistent with the authoritative UniProt subcellular location and with NDUFA11 being a multi-pass inner-membrane-arm subunit of Complex I. Correct and the most specific membrane location. Supporting Evidence: file:human/NDUFA11/NDUFA11-uniprot.txt Mitochondrion inner membrane |
| GO:0006120 mitochondrial electron transport, NADH to ubiquinone | IEA GO_REF:0000002 | ACCEPT | Summary: InterPro-based electronic annotation to the defining biological process of Complex I (electron transport from NADH to ubiquinone). This is the complex-level process in which NDUFA11 participates as a structural subunit and is the appropriate core BP for this gene. Supporting Evidence: file:human/NDUFA11/NDUFA11-uniprot.txt Complex I functions in the transfer of electrons |
| GO:0045271 respiratory chain complex I | IEA GO_REF:0000002 | ACCEPT | Summary: InterPro-based electronic transfer of Complex I membership. Correct; redundant with the experimental IDA/IPI and phylogenetic IBA annotations to the same term. Core cellular-component annotation. |
| GO:0005515 protein binding | IPI PMID:32296183 A reference map of the human binary protein interactome. | MARK AS OVER ANNOTATED | Summary: Bare "protein binding" from a genome-wide binary yeast two-hybrid interactome screen (HuRI). In the UniProt record the reported partner is MEOX2 (a homeobox transcription factor), which is not a plausible functional partner for a Complex I inner-membrane subunit and most likely reflects a high-throughput Y2H interaction rather than a physiologically meaningful one. GO:0005515 is uninformative as a molecular function (it conveys no specific activity) and this subunit's real interactions are with other Complex I subunits within the holoenzyme. Not removed per policy (IPI protein-binding), but flagged as over-annotated. Reason: Uninformative bare protein-binding term from a high-throughput Y2H screen; the reported partner (MEOX2) is not a plausible functional partner for a Complex I membrane subunit. |
| GO:0005743 mitochondrial inner membrane | IDA PMID:28844695 Architecture of Human Mitochondrial Respiratory Megacomplex ... | ACCEPT | Summary: Direct-assay (IDA, ComplexPortal) localization to mitochondrial inner membrane, supported by the cryo-EM megacomplex structure in which NDUFA11 is assigned as a Complex I membrane-arm subunit. Correct and consistent with UniProt. Supporting Evidence: PMID:28844695 reveals the precise assignment of individual subunits of human CI |
| GO:0009060 aerobic respiration | NAS PMID:30030361 Assembly of mammalian oxidative phosphorylation complexes I-... | KEEP AS NON CORE | Summary: Complex/pathway-level biological process attributed by ComplexPortal (NAS). Aerobic respiration is a downstream consequence of Complex I function rather than the direct, subunit-specific function of NDUFA11. Correct as context but broader than this gene's core role; keep as non-core. |
| GO:0042776 proton motive force-driven mitochondrial ATP synthesis | NAS PMID:30030361 Assembly of mammalian oxidative phosphorylation complexes I-... | KEEP AS NON CORE | Summary: Complex/pathway-level process (ComplexPortal NAS). ATP synthesis is a downstream outcome of the proton-motive force generated by the respiratory chain, to which Complex I contributes, but it is not the direct function of this accessory subunit. Keep as non-core contextual annotation. |
| GO:0045271 respiratory chain complex I | IPI PMID:28844695 Architecture of Human Mitochondrial Respiratory Megacomplex ... | ACCEPT | Summary: Physical-interaction (IPI, ComplexPortal) evidence that NDUFA11 is part of respiratory chain complex I, from the cryo-EM structural assignment of human Complex I subunits. Strong direct evidence for complex membership. Core cellular-component annotation. Supporting Evidence: PMID:28844695 reveals the precise assignment of individual subunits of human CI |
| GO:0005739 mitochondrion | HTP PMID:34800366 Quantitative high-confidence human mitochondrial proteome an... | KEEP AS NON CORE | Summary: High-throughput (HTP) detection of NDUFA11 in the high-confidence human mitochondrial proteome (MitoCoP). Confirms mitochondrial localization; less specific than the inner-membrane annotations. Keep as supporting, non-core evidence. Supporting Evidence: PMID:34800366 mitochondrial high-confidence proteome of >1,100 proteins |
| GO:0045271 respiratory chain complex I | IDA PMID:12611891 The subunit composition of the human NADH dehydrogenase obta... | ACCEPT | Summary: Direct-assay (IDA) identification of NDUFA11 as a subunit of immunopurified human NADH dehydrogenase (Complex I) by mass spectrometry. Primary experimental evidence for complex membership. Core cellular-component annotation. Supporting Evidence: PMID:12611891 we can resolve and identify the human homologues of 42 polypeptides detected so far in the more extensively studied |
| GO:0045271 respiratory chain complex I | IDA PMID:27626371 Accessory subunits are integral for assembly and function of... | ACCEPT | Summary: Direct experimental evidence (gene-editing/knockout plus quantitative proteomics) that NDUFA11 is a Complex I accessory subunit whose loss affects the stability of co-module subunits. Strong support for complex membership and the assembly/stability role of this subunit. Core cellular-component annotation. Supporting Evidence: PMID:27626371 loss of each subunit affects the stability of other subunits residing in the same structural module. |
| GO:0005743 mitochondrial inner membrane | TAS Reactome:R-HSA-163217 | ACCEPT | Summary: Traceable-author (Reactome) localization to mitochondrial inner membrane, from the Complex I electron-transfer reaction event. Correct localization for this inner-membrane subunit. |
| GO:0005743 mitochondrial inner membrane | TAS Reactome:R-HSA-6799178 | ACCEPT | Summary: Reactome TAS inner-membrane localization from a Complex I biogenesis event. Correct localization; redundant with other inner-membrane annotations. |
| GO:0005743 mitochondrial inner membrane | TAS Reactome:R-HSA-6799179 | ACCEPT | Summary: Reactome TAS inner-membrane localization from a Complex I biogenesis event. Correct localization; redundant with other inner-membrane annotations. |
| GO:0005743 mitochondrial inner membrane | TAS Reactome:R-HSA-6799191 | ACCEPT | Summary: Reactome TAS inner-membrane localization from a Complex I biogenesis event. Correct localization; redundant with other inner-membrane annotations. |
| GO:0005743 mitochondrial inner membrane | TAS Reactome:R-HSA-6799196 | ACCEPT | Summary: Reactome TAS inner-membrane localization from a Complex I biogenesis event. Correct localization; redundant with other inner-membrane annotations. |
| GO:0005743 mitochondrial inner membrane | TAS Reactome:R-HSA-6799197 | ACCEPT | Summary: Reactome TAS inner-membrane localization from a Complex I biogenesis event. Correct localization; redundant with other inner-membrane annotations. |
| GO:0005743 mitochondrial inner membrane | TAS Reactome:R-HSA-6799202 | ACCEPT | Summary: Reactome TAS inner-membrane localization from a Complex I biogenesis event. Correct localization; redundant with other inner-membrane annotations. |
| GO:0005739 mitochondrion | ISS GO_REF:0000024 | KEEP AS NON CORE | Summary: Sequence-similarity-based (ISS) localization to mitochondrion, transferred from a mouse ortholog. Correct but less specific than mitochondrial inner membrane, which is separately and more precisely annotated. Keep as non-core generic localization. |
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