Enhancement of stem cell engraftment on a WHIM

Research output: Contribution to journalReview article

Abstract

WHIM (warts, hypogammaglobulinemia, infections, and myelokathexis) syndrome is a genetic autoimmune disorder that results from gain-offunction mutations in the gene encoding chemokine receptor CXCR4. A previous study characterized a patient with WHIM who underwent a chromothriptic event that resulted in spontaneous deletion of the WHIM allele in a single hematopoietic stem cell and subsequent cure of the disease. In this issue of the JCI, Gao et al. extend this work and show that Cxcl4-haplosufficient bone marrow has a selective advantage for long-term engraftment in murine WHIM models. Moreover, successful engraftment occurred without prior conditioning of recipients. Together, these results have important implications for improving hematopoietic stem/progenitor cell transplant not only for patients with WHIM but also for all patients who may require the procedure.

Original languageEnglish (US)
Pages (from-to)3240-3242
Number of pages3
JournalJournal of Clinical Investigation
Volume128
Issue number8
DOIs
StatePublished - Aug 1 2018

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Stem Cells
Hematopoietic Stem Cells
Inborn Genetic Diseases
Chemokine Receptors
Bone Marrow
Alleles
WHIM syndrome
Transplants
Mutation
Genes

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Enhancement of stem cell engraftment on a WHIM. / Broxmeyer, Hal.

In: Journal of Clinical Investigation, Vol. 128, No. 8, 01.08.2018, p. 3240-3242.

Research output: Contribution to journalReview article

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