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Pathology JIPMER 2019 8aa0bd32

In alveolar variant of rhabdomyosarcoma , the resultant fusion protein is believed to function as

A
Activated growth factor receptor
B
Chimeric transcription factor
C
Constitutively active kinase
D
Novel growth factor
High-Yield Explanation
In Alveolar rhabdomyosarcoma, there is frequently fusion of the FOXO1 gene to either the PAX3 or the PAX7 gene, rearrangements marked by the presence of (2;13) or(1;13) translocations, respectively. PAX3 is a transcription factor that initiates skeletal muscle differentiation, and it appears that the chimeric PAX3-FOXO1 fusion protein interferes with the gene expression program that drives differentiation.

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