BEAN1

Chr 16

brain expressed associated with NEDD4 1

Also known as: BEAN, SCA31

The protein encoded by this gene is one of several proteins that interact with NEDD4, a member of a family of ubiquitin-protein ligases. These proteins have PY motifs in common that bind to the WW domains of NEDD4. NEDD4 is developmentally regulated, and is highly expressed in embryonic tissues. Mutations in this gene (i.e., intronic insertions of >100 copies of pentanucleotide repeats including a (TGGAA)n sequence) are associated with spinocerebellar ataxia type 31. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, May 2010]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtSpinocerebellar ataxia 31
1
Active trials
4
Pubs (1 yr)
P/LP submissions
P/LP missense
1.68
LOEUF
Multiple*
Mechanism· predicted
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GeneReview available — BEAN1
Authoritative clinical overview · Recommended first read
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Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.68LOEUF
pLI 0.000
Z-score 0.09
OE 0.97 (0.561.68)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
1.10Z-score
OE missense 0.76 (0.650.88)
124 obs / 163.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.97 (0.561.68)
00.351.4
Missense OE?0.76 (0.650.88)
00.61.4
Synonymous OE?0.72
01.21.6
LoF obs/exp: 8 / 8.3Missense obs/exp: 124 / 163.7Syn Z: 1.79

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

BEAN1 · protein map & ClinVar variants

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