DNAJC11

Chr 1

DnaJ heat shock protein family (Hsp40) member C11

Also known as: dJ126A5.1

NCBI Gene: 55735 ENSG00000007923 UniProt: Q9NVH1 OMIM: 614827

DNAJC11 encodes a protein required for mitochondrial inner membrane organization through its association with the MICOS complex and mitochondrial outer membrane sorting assembly machinery. Mutations cause autosomal recessive mitochondrial disorders affecting multiple organ systems. This gene is highly constrained against loss-of-function variants in the general population.

OMIM ResearchSummary from RefSeq, UniProt

Involved in cristae formation. Located in MICOS complex and SAM complex. Part of MIB complex. [provided by Alliance of Genome Resources, Jun 2026]

OMIM ResearchGenerating clinical summary…

Clinical highlights

Interpreting a novel variant

This gene is strongly intolerant of loss-of-function variation in the population, so LoF variants warrant close attention. No curated mechanism annotation is available — see the mechanism card for the computational prediction and its caveats.Based on population constraint only.

Active trials

Pubs (1 yr)

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P/LP submissions

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P/LP missense

0.24

LOEUF· LoF intol.

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Mechanism