Bcor insufficiency promotes initiation and progression of myelodysplastic syndrome.

ABSTRACT: BCOR, encoding BCL-6 corepressor (BCOR), is X-linked and targeted by somatic mutations in various hematological malignancies including myelodysplastic syndrome (MDS). We previously reported that mice lacking Bcor exon 4 (Bcor ^ΔE4/y ) in the hematopoietic compartment developed NOTCH-dependent acute T-cell lymphoblastic leukemia (T-ALL). Here, we analyzed mice lacking Bcor exons 9 and 10 (Bcor ^ΔE9-10/y ), which express a carboxyl-terminal truncated BCOR that fails to interact with core effector components of polycomb repressive complex 1.1. Bcor ^ΔE9-10/y mice developed lethal T-ALL in a similar manner to Bcor ^ΔE4/y mice, whereas Bcor ^ΔE9-10/y hemat

SUBMITTER: Tara S 

PROVIDER: S-EPMC6450057 | biostudies-literature | 2018 Dec

REPOSITORIES: biostudies-literature