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Zellweger Syndrome

Disease ID: disease_node_8124

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DbxrefGARD:7917, ICD10CM:E71.510, MESH:D015211, NCI:C85239, ORDO:912, SNOMEDCT_US_2023_03_01:88469006, UMLS_CUI:C0043459
SubclassofDOID_0080377, DOID_0050737
Data SourceDOID, MESH
Synonymscerebrohepatorenal syndrome, congenital iron overload
Mesh IdD015211
Mesh LabelZellweger Syndrome
Mesh SubclassofD020739, D008107, D018901, D000015, D007674
Doid LabelZellweger syndrome
Doid DescriptionA peroxisomal biogenesis disorder that is characterized by severe neurologic dysfunction, craniofacial abnormalities, and liver dysfunction, and biochemically by the absence of peroxisome and that has_material_basis_in autosomal recessive inheritance of a mutation in the peroxisome biogenesis factor (PEX) genes. OMIM mapping confirmed by DO. [LS]. OMIM mapping confirmed by DO. [SN].
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_8124
Doid IdDOID_905
LabelZellweger Syndrome