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Megaconial Type Congenital Muscular Dystrophy

Disease ID: disease_node_17421

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DbxrefICD10CM:G71.2, MIM:602541, ORDO:280671
SubclassofDOID_0050737, DOID_0050557
Data SourceDOID
Synonymscongenital megaconial myopathy, congenital muscular dystrophy due to phosphatidylcholine biosynthesis defect, congenital muscular dystrophy with mitochondrial structural abnormalities, megaconial congenital muscular dystrophy
Doid Labelmegaconial type congenital muscular dystrophy
Doid DescriptionA congenital muscular dystrophy characterized by autosomal recessive inheritance of early-onset muscle wasting and intellectual disability with enlarged mitochondria that are more prevalent towards the periphery of the fibers that has_material_basis_in homozygous or compound heterozygous mutation in the CHKB gene on chromosome 22q13.
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_17421
Doid IdDOID_0110632
Disease Has Basis InHP_0001197
LabelMegaconial Type Congenital Muscular Dystrophy