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Chromosome 1P36.33 Duplication Syndrome

Disease ID: disease_node_19219

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DbxrefMIM:618815, ORDO:656279, UMLS_CUI:C5394150
SubclassofDOID_0050736, DOID_0060429
Data SourceDOID
SynonymsCHROMOSOME 1p36.33 DUPLICATION SYNDROME, ATAD3 GENE CLUSTER, AUTOSOMAL DOMINANT
Doid Labelchromosome 1p36.33 duplication syndrome
Doid DescriptionA chromosomal duplication syndrome characterized by cardiomyopathy, corneal clouding or cataracts, hyperlactacidemia, and perinatal death that has_material_basis_in heterozygous duplication within the ATAD3 gene cluster, including the ATAD3A, ATAD3B, and ATAD3C genes, on chromosome 1p36.33 resulting in ATAD3A/ATAD3C gene fusion. Hypotonia, encephalopathy, seizures, and white matter abnormalities are also common.
Has Material Basis InGENO_0000147
Disease Node Iddisease_node_19219
Doid IdDOID_0070470
LabelChromosome 1P36.33 Duplication Syndrome