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Cortisone Reductase Deficiency 1

Disease ID: disease_node_17241

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DbxrefMIM:604931, NCI:C131849
SubclassofDOID_0090139, DOID_0050737
Data SourceDOID
SynonymsCORTRD1
Doid Labelcortisone reductase deficiency 1
Doid DescriptionA cortisone reductase deficiency that is characterized by failure to regenerate cortisol via the enzyme 11-beta-hydroxysteroid dehydrogenase which requires NADPH regeneration by hexose-6-phosphate dehydrogenase, resulting in ACTH-mediated adrenal hyperandrogenism. It has_material_basis_in autosomal recessive inheritance of homozygous or compound heterozygous mutation in the hexose-6-phosphate dehydrogenase gene (H6PD) on chromosome 1p36.
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_17241
Doid IdDOID_0090141
LabelCortisone Reductase Deficiency 1