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Carnitine Palmitoyltransferase I Deficiency

Disease ID: disease_node_16968

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DbxrefGARD:1120, ICD10CM:E71.3, MIM:255120, ORDO:156
SubclassofDOID_3146
Data SourceDOID
SynonymsCPT I deficiency, CPT1A deficiency, L-CPT1 deficiency, carnitine palmitoyl transferase 1A deficiency, carnitine palmitoyl transferase IA deficiency, hepatic CPT deficiency type I, hepatic carnitine palmitoyl transferase 1 deficiency, hepatic carnitine palmitoyl transferase I deficiency
Doid Labelcarnitine palmitoyltransferase I deficiency
Doid DescriptionA lipid metabolism disorder that is characterized by impaired mitochondrial oxidation of long chain fatty acids in the liver and kidneys resulting in episodes of illness- or fasting-induced hypoketotic hypoglycemia, and has_material_basis_in autosomal recessive inheritance of homozygous or compound heterozygous mutation in the carnitine palmitoyltransferase 1A gene (CPT1A) on chromosome 11q13.
Disease Node Iddisease_node_16968
Doid IdDOID_0090129
Disease Has Basis InSO_0001537
LabelCarnitine Palmitoyltransferase I Deficiency