SGCA

Chr 17AR

sarcoglycan alpha

Also known as: 50DAG, ADL, DAG2, DMDA2, LGMD2D, LGMDR3, SCARMD1, adhalin

This gene encodes a component of the dystrophin-glycoprotein complex (DGC), which is critical to the stability of muscle fiber membranes and to the linking of the actin cytoskeleton to the extracellular matrix. Its expression is thought to be restricted to striated muscle. Mutations in this gene result in type 2D autosomal recessive limb-girdle muscular dystrophy. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2008]

GeneReviewsOMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Muscular dystrophy, limb-girdle, autosomal recessive 3MIM #608099
AR

Clinical highlights

Gene-disease validity (ClinGen)
autosomal recessive limb-girdle muscular dystrophy · ARDefinitivesufficient evidence for diagnostic panels
0
Active trials
31
Pubs (1 yr)
P/LP submissions
P/LP missense
0.79
LOEUF
GOF
Mechanism· predicted
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GeneReview available — SGCA
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.79LOEUF
pLI 0.003
Z-score 2.19
OE 0.42 (0.240.79)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.68Z-score
OE missense 0.88 (0.790.98)
214 obs / 243.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.42 (0.240.79)
00.351.4
Missense OE?0.88 (0.790.98)
00.61.4
Synonymous OE?0.99
01.21.6
LoF obs/exp: 7 / 16.7Missense obs/exp: 214 / 243.7Syn Z: 0.11

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SGCA · 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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Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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