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Metadata
ID DOID:0060273
PURL http://purl.obolibrary.org/obo/DOID_0060273 Copy
Name pontocerebellar hypoplasia type 4
Definition A pontocerebellar hypoplasia that is characterized by progressive microcephaly, hypertonia, myoclonus, seizure and early lethality, has_material_basis_in autosomal recessive inheritance of mutation in the TSEN54 gene.
https://pubmed.ncbi.nlm.nih.gov/18711368/
Xrefs

GARD:343

MESH:C536716

MIM:225753

ORDO:166063

SNOMEDCT_US_2025_09_01:718608006

UMLS_CUI:C1856974

Subsets

DO_rare_slim

Parent Relationships

is_a pontocerebellar hypoplasia

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