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Arc Syndrome

Disease ID: disease_node_20360

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DbxrefMIM:PS208085, ORDO:2697
SubclassofDOID_225, DOID_0050737
Data SourceDOID
SynonymsARTHROGRYPOSIS, RENAL DYSFUNCTION, AND CHOLESTASIS, Arthrogryposis, renal dysfunction and cholestasis (ARC) syndrome, Arthrogryposis-renal dysfunction-cholestasis
Doid LabelARC syndrome
Doid DescriptionA syndrome that is characterized by congenital joint contractures, renal tubular dysfunction, cholestasis, severe failure to thrive, ichthyosis and a defect in platelet alpha-granule biogenesis and that has_material_basis_in homozygous or compound heterozygous mutation in the VPS33B gene or homozygous or compound heterozygous mutation in the VIPAR gene on chromosome 14q24.3.
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_20360
Doid IdDOID_0050763
LabelArc Syndrome