SNAPIN

Chr 1AR

SNAP associated protein

Also known as: BLOC1S7, BLOS7, BORCS3, NEDBAC, SNAPAP

SNAPIN encodes a component of the BLOC-1 complex required for biogenesis of lysosome-related organelles and synaptic vesicle trafficking, and also participates in SNARE-mediated neurotransmitter release at synapses. Mutations cause autosomal recessive neurodevelopmental disorder with structural brain abnormalities and craniofacial abnormalities. The gene shows low constraint against loss-of-function variants, consistent with its recessive inheritance pattern.

OMIMResearchSummary from RefSeq, OMIM, UniProt
MultiplemechanismARLOEUF 1.441 OMIM phenotype
Clinical SummarySNAPIN
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.44LOEUF
pLI 0.002
Z-score 0.77
OE 0.69 (0.361.44)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.88Z-score
OE missense 0.73 (0.590.90)
59 obs / 81.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.69 (0.361.44)
00.351.4
Missense OE0.73 (0.590.90)
00.61.4
Synonymous OE1.15
01.21.6
LoF obs/exp: 5 / 7.2Missense obs/exp: 59 / 81.3Syn Z: -0.69
DN
0.76top 25%
GOF
0.6932th %ile
LOF
0.3649th %ile

This gene has evidence for multiple mechanisms of pathogenicity (dominant-negative and gain-of-function). Both the Badonyi & Marsh prediction and the broader genomic evidence point to dominant-negative as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

DNprediction above median
GOFprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

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

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SNAPIN · protein map & ClinVar variants

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

3D Protein StructureAlphaFold

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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