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BTHLM1A

Chr 21AD

collagen type VI alpha 1 chain

Also known as: BTHLM1, BTHLM1A, OPLL, UCHMD1, UCHMD1A

The protein encoded by this gene is the alpha 1 subunit of type VI collagen, which forms heterotrimers that are major structural components of microfibrils in the extracellular matrix. Mutations cause Bethlem myopathy 1A, an autosomal dominant muscle disorder. This gene has an associated GeneReviews entry providing comprehensive clinical guidance.

GeneReviewsOMIMResearchSummary from RefSeq, OMIM
AD1 OMIM phenotype
📖
GeneReview available — BTHLM1A
Authoritative clinical overview · Recommended first read
Open GeneReview ↗
Some data sources returned errors (2)

ensembl: Error: Ensembl fetch failed: 400 for https://rest.ensembl.org/lookup/symbol/homo_sapiens/BTHLM1A?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

BTHLM1A · 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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No publications found for BTHLM1A