Congenital myopathy 2a, typical, autosomal dominant
Congenital myopathy 2a, typical, autosomal dominant
Definition
An inherited myopathy caused by mutations in the ACTA1 gene, encoding actin, alpha skeletal muscle. The phenotype is highly variable, and as such attempts at classification by clinical features is not optimal. Generally, affected individuals have generalized muscle weakness, typically involving proximal muscles, the face, bulbar and respiratory muscles.
Also known as ACTA1 nemaline myopathy, actin accumulation myopathy, actin accumulation myopathy (disorder), actin myopathy, CMYO2A, congenital myopathy 2a, typical, autosomal dominant, congenital myopathy with excess of thin filaments, nemaline myopathy caused by mutation in ACTA1, nemaline myopathy type 3 — per MONDO
Also identified as
- DOID 0110927 per MONDO
- MESH C579880 per MONDO
- MESH C580202 per MONDO
- NCIT C129870 per MONDO
- OMIM 161800 per MONDO
- Orphanet 98904 per MONDO
- SCTID 702349003 per MONDO
- UMLS C3711389 per MONDO
Affected Organs
| Organ | Involvement | Source |
|---|---|---|
| Musculature | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Musculature | Disease Has Primary Anatomic Site | NCIT · CC BY 4.0 |
| Musculoskeletal system | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Musculoskeletal system | Disease Has Primary Anatomic Site | NCIT · CC BY 4.0 |