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Mucolipidosis Ii Alpha/Beta

Disease ID: disease_node_20506

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DbxrefGARD:6749, MIM:252500
SubclassofDOID_0080488
Data SourceDOID
SynonymsI-cell disease, inclusion-cell disease, mucolipidosis II
Doid Labelmucolipidosis II alpha/beta
Doid DescriptionA mucolipidosis that is characterized by short stature, skeletal abnormalities, cardiomegaly, and developmental delay, caused by a defect in proper lysosomal enzyme phosphorylation and localization, which results in accumulation of lysosomal substrates, and that has_material_basis_in homozygous or compound heterozygous mutation in the GNPTAB gene.
Disease Node Iddisease_node_20506
Doid IdDOID_0080070
LabelMucolipidosis Ii Alpha/Beta