EMC1

Chr 1AR

ER membrane protein complex subunit 1

Also known as: CAVIPMR, KIAA0090

This protein is a component of the endoplasmic reticulum membrane protein complex (EMC) that facilitates the insertion of newly synthesized membrane proteins into ER membranes, particularly those with weakly hydrophobic transmembrane domains. Biallelic mutations cause autosomal recessive cerebellar atrophy with visual impairment and psychomotor retardation. The gene shows no loss-of-function intolerance (pLI near zero), consistent with the recessive inheritance pattern.

GeneReviewsOMIMResearchSummary from RefSeq, OMIM, UniProt
MultiplemechanismARLOEUF 1.011 OMIM phenotype
Clinical SummaryEMC1
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Gene-Disease Validity (ClinGen)
complex neurodevelopmental disorder with motor features · ARModerate

Moderate evidence — consider for supplementary testing

2 total gene-disease associations curated

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📖
GeneReview available — EMC1
Authoritative clinical overview · Recommended first read
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.01LOEUF
pLI 0.000
Z-score 1.49
OE 0.79 (0.621.01)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
1.35Z-score
OE missense 0.84 (0.780.91)
493 obs / 585.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.79 (0.621.01)
00.351.4
Missense OE0.84 (0.780.91)
00.61.4
Synonymous OE0.87
01.21.6
LoF obs/exp: 46 / 58.3Missense obs/exp: 493 / 585.0Syn Z: 1.54

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

EMC1 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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Clinical Literature
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