Dicer1 Syndrome

Alias:
Pleuro-Pulmonary Blastoma Familial Tumor Susceptibility Syndrome
Pleuropulmonary Blastoma Familial Tumor Susceptibility Syndrome
Ppb Familial Tumor Susceptibility Syndrome
Dicer1-Related Pleuropulmonary Blastoma Cancer Predisposition Syndrome
Pleuropulmonary Blastoma Familial Tumor and Dysplasia Syndrome
Pleuropulmonary Blastoma Family Tumor Susceptibility Syndrome
Pleuro-Pulmonary Blastoma Familial Tumor Susceptibility
Ppbftds
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Basic Information
Medical Symptom
Gene & Mutation
Related Drugs
Disease Model
References Literature
Dicer1 Syndrome, also known as pleuro-pulmonary blastoma familial tumor susceptibility syndrome, is related to pleuropulmonary blastoma and tumor predisposition syndrome 1. An important gene associated with Dicer1 Syndrome is DICER1 (Dicer 1, Ribonuclease III), and among its related pathways/superpathways are Fragile X syndrome and Primary ovarian insufficiency. The drugs Vincristine and Dactinomycin have been mentioned in the context of this disorder. Affiliated tissues include thyroid and lung, and related phenotypes are Increased proliferation and Decreased viability in pancreas lineage
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Basic Information

Inheritance
Age of Onset
Prevalence
Related Gene
Related Mouse Models
Reference
MALACARDS
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Unknown
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18
301
120

Medical Symptom

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Description
HPO Frequency
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Gene & Mutation

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Disease Model

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MGI
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Publications
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References Literature

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