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Congenital Disorder Of Glycosylation Iq

Disease ID: disease_node_20405

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DbxrefGARD:12397, MIM:612379, ORDO:324737
SubclassofDOID_0050737, DOID_0050570
Data SourceDOID
Synonymscongenital disorder of glycosylation 1q
Doid Labelcongenital disorder of glycosylation Iq
Doid DescriptionA congenital disorder of glycosylation I that is characterized by a highly variable phenotype typically presenting with severe visual impairment, variable ocular anomalies (such as optic nerve hypoplasia/atrophy, iris and optic nerve coloboma, congenital cataract, glaucoma), intellectual disability, cerebellar abnormalities, nystagmus, hypotonia, ataxia, and/or ichthyosiform skin lesions and has_material_basis_in homozygous or compound heterozygous mutation in the SRD5A3 gene on chromosome 4q12.
Has PhenotypeHP_0000609, HP_0006817, HP_0001272, HP_0001251, HP_0001252, HP_0000505, HP_0000612, HP_0000639, HP_0007431
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_20405
Doid IdDOID_0080568
Disease Has Basis InHP_0001197
LabelCongenital Disorder Of Glycosylation Iq