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FSHD2

Chr 18Digenic dominant

structural maintenance of chromosomes flexible hinge domain containing 1

Also known as: BAMS, FSHD2

This protein contains a hinge region domain characteristic of the SMC (structural maintenance of chromosomes) family. Mutations cause facioscapulohumeral muscular dystrophy 2, a progressive muscle disorder primarily affecting facial, shoulder, and upper arm muscles. The condition follows digenic dominant inheritance, requiring mutations in both this gene and another gene for disease manifestation.

OMIMResearchSummary from RefSeq, OMIM
Digenic dominant1 OMIM phenotype
Clinical SummaryFSHD2
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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available
Some data sources returned errors (2)

ensembl: Error: Ensembl fetch failed: 400 for https://rest.ensembl.org/lookup/symbol/homo_sapiens/FSHD2?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

FSHD2 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
Clinical Literature
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 5 results · since 2015Search PubMed ↗