SNTB1

Chr 8

syntrophin beta 1

Also known as: 59-DAP, A1B, BSYN2, DAPA1B, SNT2, SNT2B1, TIP-43

The encoded protein is an adapter protein that binds to and organizes the subcellular localization of membrane proteins, linking various receptors to the actin cytoskeleton and dystrophin glycoprotein complex. Mutations cause autosomal recessive myasthenia gravis with episodic apnea. This gene is highly constrained against loss-of-function variants, suggesting complete loss of protein function has severe consequences.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
5
Pubs (1 yr)
53
P/LP submissions
0%
P/LP missense
0.69
LOEUF
Mechanism
Clinical SummarySNTB1
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
53 unique Pathogenic / Likely Pathogenic· 101 VUS of 164 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.69LOEUF
pLI 0.001
Z-score 2.68
OE 0.39 (0.240.69)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
-0.92Z-score
OE missense 1.15 (1.051.26)
345 obs / 300.2 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.39 (0.240.69)
00.351.4
Missense OE1.15 (1.051.26)
00.61.4
Synonymous OE0.81
01.21.6
LoF obs/exp: 9 / 22.8Missense obs/exp: 345 / 300.2Syn Z: 1.72

ClinVar Variant Classifications

164 submitted variants in ClinVar

Classification Summary

Pathogenic53
VUS101
53
Pathogenic
101
VUS

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
53
0
53
Likely Pathogenic
0
0
0
0
0
VUS
0
99
2
0
101
Likely Benign
0
0
0
0
0
Benign
0
0
0
0
0
Total099550154

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

SNTB1 · 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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