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Dicer1 Syndrome

Disease ID: disease_node_20090

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DbxrefMIM:601200, ORDO:284343
SubclassofDOID_0050736, DOID_225
Data SourceDOID
SynonymsPPB familial tumor susceptibility syndrome, Pleuro-pulmonary blastoma familial tumor susceptibility, Pleuro-pulmonary blastoma familial tumor susceptibility syndrome, Pleuropulmonary blastoma familial tumor susceptibility syndrome
Doid LabelDICER1 syndrome
Doid DescriptionA syndrome that is characterized by an increased risk of developing pleuropulmonary blastoma, multinodular goiter, ovarian Sertoli-Leydig cell tumors, and/or other types of tumors, and that has_material_basis_in heterozygous mutation in the DICER1 gene on chromosome 14q32. Mutations of the gene encoding the endoribonuclease, Dicer, disrupts the biogenesis and processing of miRNAs with subsequent disruption in control of gene expression.
Has Material Basis InGENO_0000147
Disease Node Iddisease_node_20090
Doid IdDOID_0081063
LabelDicer1 Syndrome