Journal
CURRENT OPINION IN HEMATOLOGY
Volume 29, Issue 2, Pages 92-102Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/MOH.0000000000000699
Keywords
7q; contiguous gene syndrome; CUX1; monosomy 7; myeloid neoplasia
Categories
Funding
- NIH [T32 GM007281, R01 HL142782, R01 CA231880]
- American Cancer Society [132457-RSG-18-171-01-LIB]
- NIH/NCI [P30 CA014599]
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Loss of chromosome 7 is associated with adverse-risk myeloid malignancy. CUX1 is a critical tumor suppressor gene located within a commonly deleted segment of chromosome arm 7q. Studies have also identified other genes encoded on chromosome 7 as myeloid tumor suppressors, implicating chromosome 7 deletions in disease pathogenesis.
Purpose of review Loss of chromosome 7 has long been associated with adverse-risk myeloid malignancy. In the last decade, CUX1 has been identified as a critical tumor suppressor gene (TSG) located within a commonly deleted segment of chromosome arm 7q. Additional genes encoded on 7q have also been identified as bona fide myeloid tumor suppressors, further implicating chromosome 7 deletions in disease pathogenesis. This review will discuss the clinical implications of del(7q) and CUX1 mutations, both in disease and clonal hematopoiesis, and synthesize recent literature on CUX1 and other chromosome 7 TSGs. Recent findings Two major studies, including a new mouse model, have been published that support a role for CUX1 inactivation in the development of myeloid neoplasms. Additional recent studies describe the cellular and hematopoietic effects from loss of the 7q genes LUC7L2 and KMT2C/MLL3, and the implications of chromosome 7 deletions in clonal hematopoiesis. Mounting evidence supports CUX1 as being a key chromosome 7 TSG. As 7q encodes additional myeloid regulators and tumor suppressors, improved models of chromosome loss are needed to interrogate combinatorial loss of these critical 7q genes.
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