TIMM50

Chr 19AR

translocase of inner mitochondrial membrane 50

Also known as: MGCA9, TIM50, TIM50L

This gene encodes a subunit of the TIM23 inner mitochondrial membrane translocase complex. The encoded protein functions as the receptor subunit that recognizes the mitochondrial targeting signal, or presequence, on protein cargo that is destined for the mitochondrial inner membrane and matrix. This protein may also play a role in maintaining the membrane permeability barrier, and knockdown of this gene in human cells results in the release of cytochrome c and apoptosis. [provided by RefSeq, Jul 2016]

OMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

3-methylglutaconic aciduria, type IXMIM #617698
AR

Clinical highlights

Gene-disease validity (ClinGen)
3-methylglutaconic aciduria type 9 · ARDefinitivesufficient evidence for diagnostic panels
0
Active trials
3
Pubs (1 yr)
P/LP submissions
P/LP missense
0.65
LOEUF
Mechanism

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.65LOEUF
pLI 0.002
Z-score 2.88
OE 0.37 (0.220.65)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.04Z-score
OE missense 0.99 (0.901.09)
291 obs / 293.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.37 (0.220.65)
00.351.4
Missense OE?0.99 (0.901.09)
00.61.4
Synonymous OE?1.13
01.21.6
LoF obs/exp: 9 / 24.3Missense obs/exp: 291 / 293.0Syn Z: -1.15

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TIMM50 · 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.

Search ClinicalTrials.gov →