CHRNG

Chr 2AR

cholinergic receptor nicotinic gamma subunit

Also known as: ACHRG

The gamma subunit forms part of the fetal muscle-type acetylcholine receptor pentamer at the neuromuscular junction, functioning in neuromuscular organogenesis and ligand binding. Mutations cause Escobar syndrome and lethal multiple pterygium syndrome through autosomal recessive inheritance. The pathogenic mechanism involves gain-of-function effects that disrupt normal receptor localization in cell membranes.

Summary from RefSeq, OMIM, UniProt, Mechanism
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Primary Disease Associations & Inheritance

Escobar syndromeMIM #265000
AR
Multiple pterygium syndrome, lethal typeMIM #253290
AR
0
Active trials
9
Pubs (1 yr)
0
P/LP submissions
P/LP missense
1.32
LOEUF
LOF
Mechanism· G2P
Clinical SummaryCHRNG
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Gene-Disease Validity (ClinGen)
CHRNG-associated hypo-akinesia disorder of prenatal onset · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📖
GeneReview available — CHRNG
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
1.32LOEUF
pLI 0.000
Z-score 0.18
OE 0.97 (0.721.32)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.12Z-score
OE missense 1.02 (0.931.12)
310 obs / 304.2 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.97 (0.721.32)
00.351.4
Missense OE1.02 (0.931.12)
00.61.4
Synonymous OE1.00
01.21.6
LoF obs/exp: 29 / 30.0Missense obs/exp: 310 / 304.2Syn Z: 0.03
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveCHRNG-related multiple pterygium syndrome, Escobar variantLOFAR

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.7131th %ile
GOF
0.83top 10%
LOF
0.2678th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

CHRNG · 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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Clinical Literature
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