SGCG

Chr 13AR

sarcoglycan gamma

Also known as: 35DAG, A4, DAGA4, DMDA, DMDA1, LGMD2C, LGMDR5, MAM

This gene encodes gamma-sarcoglycan, one of several sarcolemmal transmembrane glycoproteins that interact with dystrophin. The dystrophin-glycoprotein complex (DGC) spans the sarcolemma and is comprised of dystrophin, syntrophin, alpha- and beta-dystroglycans and sarcoglycans. The DGC provides a structural link between the subsarcolemmal cytoskeleton and the extracellular matrix of muscle cells. Defects in the encoded protein can lead to early onset autosomal recessive muscular dystrophy, in particular limb-girdle muscular dystrophy, type 2C (LGMD2C). [provided by RefSeq, Oct 2008]

GeneReviewsOMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Muscular dystrophy, limb-girdle, autosomal recessive 5MIM #253700
AR

Clinical highlights

Gene-disease validity (ClinGen)
autosomal recessive limb-girdle muscular dystrophy · ARDefinitivesufficient evidence for diagnostic panels
1
Active trials
12
Pubs (1 yr)
P/LP submissions
P/LP missense
1.05
LOEUF
Multiple*
Mechanism· predicted
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GeneReview available — SGCG
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?
1.05LOEUF
pLI 0.000
Z-score 1.44
OE 0.60 (0.361.05)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-0.13Z-score
OE missense 1.03 (0.911.17)
168 obs / 163.2 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.60 (0.361.05)
00.351.4
Missense OE?1.03 (0.911.17)
00.61.4
Synonymous OE?1.18
01.21.6
LoF obs/exp: 9 / 15.0Missense obs/exp: 168 / 163.2Syn Z: -1.14

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SGCG · protein map & ClinVar variants

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

VCEP specificationsLimb Girdle Muscular DystrophyReleased
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