DNAJC19

Chr 3

DnaJ heat shock protein family (Hsp40) member C19

Also known as: PAM18, TIM14, TIMM14

The protein encoded by this gene is thought to be part of a complex involved in the ATP-dependent transport of transit peptide-containing proteins from the inner cell membrane to the mitochondrial matrix. Defects in this gene are a cause of 3-methylglutaconic aciduria type 5 (MGA5), also known as dilated cardiomyopathy with ataxia (DCMA). Alternative splicing of this gene results in multiple transcript variants. Related pseudogenes have been identified on chromosomes 1, 2, 6, 10, 14 and 19. [provided by RefSeq, Jan 2012]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProt3-methylglutaconic aciduria 5

Clinical highlights

Gene-disease validity (ClinGen)
3-methylglutaconic aciduria type 5 · ARDefinitivesufficient evidence for diagnostic panels
0
Active trials
3
Pubs (1 yr)
P/LP submissions
P/LP missense
1.31
LOEUF
DN
Mechanism· predicted
📖
GeneReview available — DNAJC19
Authoritative clinical overview · Recommended first read
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Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.31LOEUF
pLI 0.001
Z-score 0.93
OE 0.66 (0.361.31)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.88Z-score
OE missense 0.69 (0.540.89)
44 obs / 63.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.66 (0.361.31)
00.351.4
Missense OE?0.69 (0.540.89)
00.61.4
Synonymous OE?0.96
01.21.6
LoF obs/exp: 6 / 9.0Missense obs/exp: 44 / 63.7Syn Z: 0.15

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

DNAJC19 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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