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Neuronal Ceroid Lipofuscinosis 1

Disease ID: disease_node_15965

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DbxrefGARD:1219, ICD10CM:E75.4, MIM:256730, ORDO:228329
SubclassofDOID_0050737, DOID_14503
Data SourceDOID
SynonymsCLN1, neuronal ceroid lipofuscinosis 1 variable age of onset
Doid Labelneuronal ceroid lipofuscinosis 1
Doid DescriptionA neuronal ceroid lipofuscinosis that is characterized by variable age of onset of symptoms (progressive dementia, seizures, and progressive visual failure) and lipopigment pattern of granular osmiophilic deposits, and has_material_basis_in homozygous or compound heterozygous mutation in the PPT1 gene on chromosome 1p34.
Has SymptomSYMP_0000124
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_15965
Doid IdDOID_0110721
LabelNeuronal Ceroid Lipofuscinosis 1