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Progressive Familial Intrahepatic Cholestasis 5

Disease ID: disease_node_17767

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DbxrefMIM:617049, ORDO:480476
SubclassofDOID_0070221, DOID_0050737
Data SourceDOID
SynonymsNR1H4 deficiency, PFIC5
Doid Labelprogressive familial intrahepatic cholestasis 5
Doid DescriptionA progressive familial intrahepatic cholestasis characterized by autosomal recessive inheritance of intralobular cholestasis with onset in the neonatal period that has_material_basis_in mutation in the NR1H4 gene on chromosome 12q.
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_17767
Doid IdDOID_0070225
LabelProgressive Familial Intrahepatic Cholestasis 5