SERAC1

Chr 6

serine active site containing 1

The protein encoded by this gene is a phosphatidylglycerol remodeling protein found at the interface of mitochondria and endoplasmic reticula, where it mediates phospholipid exchange. The encoded protein plays a major role in mitochondrial function and intracellular cholesterol trafficking. Defects in this gene are a cause of 3-methylglutaconic aciduria with deafness, encephalopathy, and Leigh-like syndrome (MEGDEL). Two transcript variants, one protein-coding and the other non-protein coding, have been found for this gene. [provided by RefSeq, Aug 2012]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProt3-methylglutaconic aciduria with deafness, encephalopathy, and Leigh-like syndrome

Clinical highlights

Gene-disease validity (ClinGen)
Leigh syndrome · ARDefinitivesufficient evidence for diagnostic panels
Interpreting a novel variant
Loss of function is the curated mechanism (Gene2Phenotype), though the gene is not strongly LoF-constrained in the population — weigh truncating variants against that tolerance.Curated gene-level mechanism — a prior for triage, not a per-variant call.
0
Active trials
14
Pubs (1 yr)
P/LP submissions
P/LP missense
0.63
LOEUF
LOF
Mechanism· G2P
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GeneReview available — SERAC1
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?
0.63LOEUF
pLI 0.000
Z-score 3.39
OE 0.41 (0.280.63)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
1.49Z-score
OE missense 0.78 (0.700.86)
278 obs / 357.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.41 (0.280.63)
00.351.4
Missense OE?0.78 (0.700.86)
00.61.4
Synonymous OE?0.83
01.21.6
LoF obs/exp: 16 / 38.8Missense obs/exp: 278 / 357.3Syn Z: 1.49

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SERAC1 · 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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