DNAJC11 is a mitochondrial protein of the DnaJ/Hsp40 (DNAJC) family with an N-terminal J domain and a C-terminal family-specific beta-barrel/coiled-coil region. Its full-length isoform is a peripheral protein of the mitochondrial outer membrane, and it is a component of the mitochondrial intermembrane space bridging (MIB) complex that links the inner-membrane MICOS (mitochondrial contact site and cristae organizing system) complex with the outer-membrane SAM (sorting and assembly machinery) complex. Through these associations DNAJC11 is required for proper mitochondrial inner-membrane organization and the formation of cristae and cristae junctions. In mice, a hypomorphic splicing mutation in DnaJC11 causes motor neuron pathology with cristae disorganization, muscle weakness, lymphoid abnormalities and early lethality, establishing a link between DNAJC11 and neuromuscular disease.
| GO Term | Evidence | Action | Reason |
|---|---|---|---|
| GO:0042407 cristae formation | IBA GO_REF:0000033 | ACCEPT | Summary: Phylogenetically inferred role in cristae formation, consistent with DNAJC11's experimentally established function in mitochondrial inner-membrane/cristae organization via the MICOS/MIB complex. Reason: Cristae formation is a core biological process for DNAJC11, supported by mouse loss-of-function (cristae disorganization) and its MICOS/MIB association. Supporting Evidence: file:human/DNAJC11/DNAJC11-uniprot.txt Required for mitochondrial inner membrane organization. |
| GO:0005739 mitochondrion | IEA GO_REF:0000044 | ACCEPT | Summary: Automated transfer of mitochondrial localization, consistent with DNAJC11's established mitochondrial localization. The mitochondrial outer membrane is the more precise compartment. Reason: Mitochondrial localization is experimentally established; this is a correct but general compartment annotation. Supporting Evidence: file:human/DNAJC11/DNAJC11-uniprot.txt SUBCELLULAR LOCATION: Mitochondrion |
| GO:0005741 mitochondrial outer membrane | IEA GO_REF:0000044 | ACCEPT | Summary: Automated transfer of mitochondrial outer membrane localization, the precise compartment of the full-length DNAJC11 isoform. Reason: The full-length isoform is a peripheral mitochondrial outer membrane protein, experimentally established (PMID:25111180). Supporting Evidence: file:human/DNAJC11/DNAJC11-uniprot.txt [Isoform 1]: Mitochondrion outer membrane |
| GO:0005515 protein binding | IPI PMID:17500595 Huntingtin interacting proteins are genetic modifiers of neu... | KEEP AS NON CORE | Summary: High-throughput interaction (IntAct WITH huntingtin/HTT, P42858). The bare protein binding term records a real interaction but is uninformative. Reason: Bare protein binding from a high-throughput screen; uninformative and not part of the core MICOS/cristae function. Supporting Evidence: file:human/DNAJC11/DNAJC11-goa.tsv GO:0005515 protein binding molecular_function ECO:0000353 IPI PMID:17500595 UniProtKB:P42858 |
| GO:0005515 protein binding | IPI PMID:32296183 A reference map of the human binary protein interactome. | KEEP AS NON CORE | Summary: High-throughput interactions (IntAct WITH PM20D2 Q8IYS1, VPS52 Q8N1B4, LXN Q9BS40). The bare protein binding term is uninformative. Reason: Bare protein binding from a high-throughput screen with partners unrelated to DNAJC11's cristae/MICOS function; uninformative. Supporting Evidence: file:human/DNAJC11/DNAJC11-goa.tsv GO:0005515 protein binding molecular_function ECO:0000353 IPI PMID:32296183 UniProtKB:Q8IYS1 |
| GO:0005515 protein binding | IPI PMID:33961781 Dual proteome-scale networks reveal cell-specific remodeling... | KEEP AS NON CORE | Summary: BioPlex affinity-purification interactome capturing a DNAJC11-ARMC1 (Q9NVT9) interaction. Bare protein binding term. Reason: Bare protein binding is uninformative; retained as a recorded interaction. Supporting Evidence: file:human/DNAJC11/DNAJC11-goa.tsv GO:0005515 protein binding molecular_function ECO:0000353 IPI PMID:33961781 UniProtKB:Q9NVT9 |
| GO:0005515 protein binding | IPI PMID:35271311 OpenCell: Endogenous tagging for the cartography of human ce... | KEEP AS NON CORE | Summary: Interactome dataset capturing a DNAJC11-ARMC1 (Q9NVT9) interaction. Bare protein binding term. Reason: Bare protein binding is uninformative; retained as a recorded interaction. Supporting Evidence: file:human/DNAJC11/DNAJC11-goa.tsv GO:0005515 protein binding molecular_function ECO:0000353 IPI PMID:35271311 UniProtKB:Q9NVT9 |
| GO:0005515 protein binding | IPI PMID:40205054 Multimodal cell maps as a foundation for structural and func... | KEEP AS NON CORE | Summary: Multimodal cell-maps interactome capturing a DNAJC11-ARMC1 (Q9NVT9) interaction. Bare protein binding term. Reason: Bare protein binding is uninformative; retained as a recorded interaction. Supporting Evidence: file:human/DNAJC11/DNAJC11-goa.tsv GO:0005515 protein binding molecular_function ECO:0000353 IPI PMID:40205054 UniProtKB:Q9NVT9 |
| GO:0042407 cristae formation | IEA GO_REF:0000120 | ACCEPT | Summary: Automated transfer of the cristae formation process, consistent with experimental and phylogenetic evidence for DNAJC11's role in cristae organization. Reason: Cristae formation is a core biological process supported by loss-of-function and MICOS/MIB association. Supporting Evidence: file:human/DNAJC11/DNAJC11-uniprot.txt Required for mitochondrial inner membrane organization. |
| GO:0140275 MIB complex | IDA PMID:25997101 QIL1 is a novel mitochondrial protein required for MICOS com... | ACCEPT | Summary: DNAJC11 is a component of the mitochondrial intermembrane space bridging (MIB) complex linking MICOS and SAM, directly demonstrated. Reason: Direct experimental evidence establishes DNAJC11 as part of the MIB complex, the structural basis of its cristae-organizing function. Supporting Evidence: file:human/DNAJC11/DNAJC11-uniprot.txt The MICOS and SAM complexes together with DNAJC11 are part of a large protein complex spanning both mitochondrial membranes termed the mitochondrial intermembrane space bridging (MIB) complex. |
| GO:0005739 mitochondrion | IDA GO_REF:0000052 | ACCEPT | Summary: HPA immunofluorescence showing mitochondrial localization, consistent with DNAJC11's established mitochondrial localization. Reason: Mitochondrial localization is experimentally supported; correct but general relative to the outer-membrane annotation. Supporting Evidence: file:human/DNAJC11/DNAJC11-uniprot.txt SUBCELLULAR LOCATION: Mitochondrion |
| GO:0005741 mitochondrial outer membrane | EXP PMID:25111180 A splicing mutation in the novel mitochondrial protein DNAJC... | ACCEPT | Summary: Experimental evidence that the full-length 63 kDa DNAJC11 isoform localizes to the periphery of the mitochondrial outer membrane. Reason: Direct experimental evidence establishes the mitochondrial outer membrane as the precise compartment of the principal DNAJC11 isoform. Supporting Evidence: PMID:25111180 The full length 63 kDa isoform of human DNAJC11 was shown to localize in the periphery of the mitochondrial outer membrane |
| GO:0005739 mitochondrion | HTP PMID:34800366 Quantitative high-confidence human mitochondrial proteome an... | ACCEPT | Summary: High-throughput proteomics detection of mitochondrial localization, consistent with the established localization. Reason: Mitochondrial localization is corroborated by direct experimental evidence; correct compartment. Supporting Evidence: file:human/DNAJC11/DNAJC11-uniprot.txt SUBCELLULAR LOCATION: Mitochondrion |
| GO:0007007 inner mitochondrial membrane organization | IC PMID:26477565 Evolution and structural organization of the mitochondrial c... | ACCEPT | Summary: Curator-inferred role (from MIB complex membership) in inner mitochondrial membrane organization, consistent with the cristae-organizing function of the MICOS/MIB system. Reason: DNAJC11 is required for mitochondrial inner-membrane organization, supported by its MIB-complex membership and mouse loss-of-function phenotype. Supporting Evidence: file:human/DNAJC11/DNAJC11-uniprot.txt Required for mitochondrial inner membrane organization. |
| GO:0140275 MIB complex | HDA PMID:26477565 Evolution and structural organization of the mitochondrial c... | ACCEPT | Summary: High-throughput proteomics evidence that DNAJC11 is part of the MIB complex, consistent with the IDA annotation. Reason: MIB-complex membership is corroborated by direct and high-throughput evidence; core to DNAJC11's function. Supporting Evidence: file:human/DNAJC11/DNAJC11-uniprot.txt The MICOS and SAM complexes together with DNAJC11 are part of a large protein complex spanning both mitochondrial membranes termed the mitochondrial intermembrane space bridging (MIB) complex. |
| GO:0005739 mitochondrion | IDA PMID:25997101 QIL1 is a novel mitochondrial protein required for MICOS com... | ACCEPT | Summary: Direct experimental evidence for mitochondrial localization of DNAJC11. Reason: Mitochondrial localization is experimentally established; correct compartment. Supporting Evidence: file:human/DNAJC11/DNAJC11-uniprot.txt SUBCELLULAR LOCATION: Mitochondrion |
Loading supporting content…
Download this section (compressed HTML)Q: Does the J domain of DNAJC11 recruit a mitochondrial HSP70 (e.g. HSPA9/mortalin) to the MIB complex, and is any co-chaperone activity required for cristae organization?
Q: What are the distinct functions of the differentially localized DNAJC11 isoforms (outer membrane vs inner-membrane/IMS forms)?
Experiment: Reconstitute or immunopurify the MIB complex with wild-type versus J-domain-mutant DNAJC11 and assess MICOS/SAM assembly, cristae junction morphology (by electron microscopy), and recruitment of mitochondrial HSP70.
Experiment: Isoform-specific knockout/rescue in human cells followed by quantitative proteomics and cryo-EM tomography to define which DNAJC11 isoform supports cristae architecture and MICOS stability.
Loading supporting content…
Download this section (compressed HTML)Loading supporting content…
Download this section (compressed HTML)Loading supporting content…
Download this section (compressed HTML)