MPC1

Chr 6AR

mitochondrial pyruvate carrier 1

Also known as: BRP44L, CGI-129, MPYCD, SLC54A1

The protein encoded by this gene is part of an MPC1/MPC2 heterodimer that is responsible for transporting pyruvate into mitochondria. The encoded protein is found in the inner mitochondrial membrane. Defects in this gene are a cause of mitochondrial pyruvate carrier deficiency. Several transcript variants, some protein coding and one non-protein coding, have been found for this gene. [provided by RefSeq, Aug 2012]

GeneReviewsOMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Mitochondrial pyruvate carrier deficiencyMIM #614741
AR

Clinical highlights

Gene-disease validity (ClinGen)
mitochondrial disease · ARDefinitivesufficient evidence for diagnostic panels
0
Active trials
45
Pubs (1 yr)
P/LP submissions
P/LP missense
1.19
LOEUF
Multiple*
Mechanism· predicted
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GeneReview available — MPC1
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.19LOEUF
pLI 0.038
Z-score 1.28
OE 0.46 (0.211.19)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
1.07Z-score
OE missense 0.62 (0.480.81)
40 obs / 64.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.46 (0.211.19)
00.351.4
Missense OE?0.62 (0.480.81)
00.61.4
Synonymous OE?1.02
01.21.6
LoF obs/exp: 3 / 6.5Missense obs/exp: 40 / 64.2Syn Z: -0.09

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

MPC1 · 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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