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Title

Wolf-Hirschhorn Syndrome Candidate 1 Is Necessary for Correct Hematopoietic and B Cell Development

AuthorsCampos-Sánchez, Elena ; Deleyto-Seldas, Nerea; Cobaleda, César
Issue Date23-May-2017
CitationCell Reports 19: 1586- 1601 (2017)
AbstractImmunodeficiency is one of the most important causes of mortality associated with Wolf-Hirschhorn syndrome (WHS), a severe rare disease originated by a deletion in chromosome 4p. The WHS candidate 1 (WHSC1) gene has been proposed as one of the main genes responsible for many of the alterations in WHS, but its mechanism of action is still unknown. Here, we present in vivo genetic evidence showing that Whsc1 plays an important role at several points of hematopoietic development. Particularly, our results demonstrate that both differentiation and function of Whsc1-deficient B cells are impaired at several key developmental stages due to profound molecular defects affecting B cell lineage specification, commitment, fitness, and proliferation, demonstrating a causal role for WHSC1 in the immunodeficiency of WHS patients.
URIhttp://hdl.handle.net/10261/166720
DOIhttp://dx.doi.org/10.1016/j.celrep.2017.04.069
Identifiersdoi: 10.1016/j.celrep.2017.04.069
issn: 2211-1247
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