VAPB

Chr 20AD

VAMP associated protein B and C

Also known as: ALS8, VAMP-B, VAP-B

VAPB encodes an endoplasmic reticulum-anchored protein that mediates contact sites between the ER and endosomes, participates in the unfolded protein response, and regulates cellular calcium homeostasis. Mutations cause late-onset motor neuron diseases including amyotrophic lateral sclerosis 8 and spinal muscular atrophy (Finkel type), with inheritance following an autosomal dominant pattern. The gene shows moderate constraint against loss-of-function variants (LOEUF 0.559).

OMIMResearchSummary from RefSeq, OMIM, UniProt
MultiplemechanismADLOEUF 0.562 OMIM phenotypes
Clinical SummaryVAPB
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Gene-Disease Validity (ClinGen)
amyotrophic lateral sclerosis type 8 · ADDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.58) — some intolerance to loss-of-function variants.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.56LOEUF
pLI 0.581
Z-score 2.56
OE 0.18 (0.070.56)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.25Z-score
OE missense 0.70 (0.590.83)
94 obs / 135.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.18 (0.070.56)
00.351.4
Missense OE0.70 (0.590.83)
00.61.4
Synonymous OE0.88
01.21.6
LoF obs/exp: 2 / 11.3Missense obs/exp: 94 / 135.0Syn Z: 0.68
DN
0.6455th %ile
GOF
0.5170th %ile
LOF
0.49top 25%

This gene has evidence for multiple mechanisms of pathogenicity (dominant-negative and loss-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 · 1 literature citation
LOF1 literature citation

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.

Literature Evidence

DNOne such mutation, P56S, was identified in human VAPB that behaves in a dominant negative manner, sequestering wild type protein into cytoplasmic inclusions.PMID:25361581
LOFA mutant, aggregation-prone, form of VAPB (P56S) is linked to a dominantly inherited form of amyotrophic lateral sclerosis; however, it has been unclear whether its pathogenicity is due to toxic gain of function, to negative dominance, or simply to insufficient levels of the wild-type protein producPMID:30745341

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

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

VAPB · 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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Full-Text Mentions
NLP-detected gene mentions in article bodies · via PubTator3
PubTator3
Top 5 full-text resultsSearch PubTator3 ↗