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CHEDDA

Chr 12AD

atrophin 1

Also known as: B37, CHEDDA, D12S755E, DRPLA, HRS, NOD

The encoded protein contains serine repeats and alternating acidic-basic amino acid regions, with expansion of CAG/CAA trinucleotide repeats from normal 7-35 copies to pathogenic 49-93 copies causing dentatorubral pallidoluysian atrophy (DRPLA). DRPLA presents with cerebellar ataxia, myoclonic epilepsy, choreoathetosis, and dementia, while other mutations cause congenital hypotonia, epilepsy, developmental delay, and digital anomalies. Inheritance is autosomal dominant.

GeneReviewsOMIMResearchSummary from RefSeq, OMIM
AD1 OMIM phenotype
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GeneReview available — CHEDDA
Authoritative clinical overview · Recommended first read
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Some data sources returned errors (2)

ensembl: Error: Ensembl fetch failed: 400 for https://rest.ensembl.org/lookup/symbol/homo_sapiens/CHEDDA?content-type=application/json&expand=1

gnomad: Error: Gene not found

Population Genetics & Constraint

Constraint data not available from gnomAD.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

CHEDDA · protein map & ClinVar variants

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

3D Protein StructureAlphaFold

Clinical Trials

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Clinical Literature
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