VWA5A

Chr 11

von Willebrand factor A domain containing 5A

Also known as: BCSC-1, BCSC1, LOH11CR2A

The protein encoded by this gene localizes to the nucleoplasm and may function as a tumor suppressor. Mutations cause neurodevelopmental disorders, though the specific phenotypes and inheritance patterns are not well-established in the provided data. This gene appears to be highly tolerant to loss-of-function variants based on constraint metrics.

Summary from RefSeq, UniProt
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0
Active trials
2
Pubs (1 yr)
0
P/LP submissions
P/LP missense
1.46
LOEUF
DN
Mechanism· predicted
Clinical SummaryVWA5A
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
1.46LOEUF
pLI 0.000
Z-score -0.71
OE 1.13 (0.881.46)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.60Z-score
OE missense 1.08 (1.001.17)
466 obs / 431.0 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.13 (0.881.46)
00.351.4
Missense OE1.08 (1.001.17)
00.61.4
Synonymous OE0.89
01.21.6
LoF obs/exp: 41 / 36.4Missense obs/exp: 466 / 431.0Syn Z: 1.10
DN
0.6745th %ile
GOF
0.6052th %ile
LOF
0.3261th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction 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

VWA5A · 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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