Article

Temporal order of RNase IIIb and loss-of-function mutations during development determines phenotype in pleuropulmonary blastoma / DICER1 syndrome: a unique variant of the two-hit tumor suppression model

2018-01-12

Abstract excerpt

Pleuropulmonary blastoma (PPB) is the most frequent pediatric lung tumor and often the first indication of a pleiotropic cancer predisposition, DICER1 syndrome, comprising a range of other individually rare, benign and malignant tumors of childhood and early adulthood. The genetics of DICER1 -associated tumorigenesis are unusual in that tumors typically bear neomorphic missense mutations at one of five specif...

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Literature Corpus work: d405f6d4-cd2c-58ca-ab74-3966ad8eaa6a
DOI: 10.12688/f1000research.6746.2

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