SGCB

Chr 4AR

sarcoglycan beta

Also known as: A3b, LGMD2E, LGMDR4, SGC

This gene encodes a member of the sarcoglycan family. Sarcoglycans are transmembrane components in the dystrophin-glycoprotein complex which help stabilize the muscle fiber membranes and link the muscle cytoskeleton to the extracellular matrix. Mutations in this gene have been associated with limb-girdle muscular dystrophy.[provided by RefSeq, Oct 2008]

GeneReviewsOMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Muscular dystrophy, limb-girdle, autosomal recessive 4MIM #604286
AR

Clinical highlights

Gene-disease validity (ClinGen)
autosomal recessive limb-girdle muscular dystrophy · ARDefinitivesufficient evidence for diagnostic panels
0
Active trials
16
Pubs (1 yr)
P/LP submissions
P/LP missense
1.47
LOEUF
DN
Mechanism· predicted
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GeneReview available — SGCB
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?
1.47LOEUF
pLI 0.000
Z-score 0.42
OE 0.87 (0.531.47)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-0.00Z-score
OE missense 1.00 (0.891.13)
178 obs / 177.8 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.87 (0.531.47)
00.351.4
Missense OE?1.00 (0.891.13)
00.61.4
Synonymous OE?1.16
01.21.6
LoF obs/exp: 10 / 11.5Missense obs/exp: 178 / 177.8Syn Z: -0.98

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SGCB · 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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