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FSHD3

Chr 1Digenic recessive

ligand dependent nuclear receptor interacting factor 1

Also known as: C1orf103, FSHD3, HBiX1, RIF1

This protein binds nuclear retinoic acid receptors and is involved in X chromosome inactivation, localizing to centriolar satellites, telomeric chromosome regions, and the nuclear lumen. Mutations cause facioscapulohumeral muscular dystrophy 3, a progressive muscle disorder affecting facial, shoulder, and upper arm muscles. The condition follows digenic recessive inheritance, requiring mutations in two different genes to manifest disease.

OMIMResearchSummary from RefSeq, OMIM
Digenic recessive1 OMIM phenotype
Some data sources returned errors (2)

ensembl: Error: Ensembl fetch failed: 400 for https://rest.ensembl.org/lookup/symbol/homo_sapiens/FSHD3?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

FSHD3 · 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

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