RTICC International Guest Seminar- Prof. Seth Corey

Prof. Seth Corey
Case Western Reserve University, Case Comprehensive Cancer Center, Department of Pediatric Hematology & Oncology, Cleveland Clinic

“P53-Dependent and Independent Stress Responses in Hematopoietic Ribosomopathy”

Shwachman-Diamond syndrome is a bone marrow failure/leukemia predisposition syndrome. Almost all of the cases of the syndrome result from ballelic mutations in SBDS, a ribosomal assembly factor. We have generated sbds-null zebrafish models of the syndrome, which phenocopy much of the syndrome. Genetic, biochemical, and organismal models reveal involvement of both p53-dependent and independent stress responses