Defective DNA damage repair leads to frequent catastrophic genomic events in murine and human tumors.

Ratnaparkhe, Manasi; Wong, John K L; Wei, Pei-Chi; Hlevnjak, Mario; Kolb, Thorsten; Simovic, Milena; Haag, Daniel; Paul, Yashna et al. · Nat Commun · 2018

basic_science · Level V

Where this comes from

Abstract

Chromothripsis and chromoanasynthesis are catastrophic events leading to clustered genomic rearrangements. Whole-genome sequencing revealed frequent complex genomic rearrangements (n = 16/26) in brain tumors developing in mice deficient for factors involved in homologous-recombination-repair or non-homologous-end-joining. Catastrophic events were tightly linked to Myc/Mycn amplification, with increased DNA damage and inefficient apoptotic response already observable at early postnatal stages. Inhibition of repair processes and comparison of the mouse tumors with human medulloblastomas (n = 68) and glioblastomas (n = 32) identified chromothripsis as associated with MYC/MYCN gains and with DNA repair deficiencies, pointing towards therapeutic opportunities to target DNA repair defects in tumors with complex genomic rearrangements.

Medical subject headings