Hematopoietic stem cell dysfunction underlies the progressive lymphocytopenia in XLF/Cernunnos deficiency-Reference-Cited by-同舟云学术

Hematopoietic stem cell dysfunction underlies the progressive lymphocytopenia in XLF/Cernunnos deficiency

Published:2014-09-04 Issue:10 Volume:124 Page:1622-1625
ISSN:0006-4971
Container-title:Blood
language:en
Short-container-title:

Author:

Avagyan Serine¹²³,Churchill Michael³⁴,Yamamoto Kenta²³⁵,Crowe Jennifer L.²³⁵,Li Chen²,Lee Brian J.²,Zheng Tian⁶,Mukherjee Siddhartha⁴,Zha Shan¹²

Affiliation:

1. Morgan Stanley Children’s Hospital of New York-Presbyterian,

2. Department of Pediatrics, Institute for Cancer Genetics,

3. Herbert Irving Comprehensive Cancer Research Center,

4. Division of Oncology, Department of Medicine, and

5. Pathobiology Graduate Program, College for Physicians and Surgeons, Columbia University Medical Center, New York, NY; and

6. Department of Statistics, Columbia University, New York, NY

Abstract

Key Points XLF-deficient mice recapitulate the lymphocytopenia of XLF-deficient patients. Premature aging of hematopoietic stem cells underlies the severe and progressive lymphocytopenia in XLF-deficient mice.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Link

http://ashpublications.org/blood/article-pdf/124/10/1622/1379991/1622.pdf

Reference23 articles.

1. Cernunnos, a novel nonhomologous end-joining factor, is mutated in human immunodeficiency with microcephaly.;Buck;Cell,2006

2. Nonhomologous end joining and V(D)J recombination require an additional factor.;Dai;Proc Natl Acad Sci USA,2003

3. Lymphocyte-specific compensation for XLF/cernunnos end-joining functions in V(D)J recombination.;Li;Mol Cell,2008

4. Cernunnos deficiency reduces thymocyte life span and alters the T cell repertoire in mice and humans.;Vera;Mol Cell Biol,2013

5. Defective DNA repair and increased genomic instability in Cernunnos-XLF-deficient murine ES cells.;Zha;Proc Natl Acad Sci USA,2007

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