Decreased immunoglobulin class switching in Nijmegen breakage syndrome due to the DNA repair defect

Human Immunology

Volumn 62, Issue 12, 2001, Pages 1324-1327

  Van Engelen, B G M ^a,b   Hiel, J A P ^a   Gabreels F J M ^a   Van Den Heuvel, L P W J ^a   Van Gent, D C ^c   Weemaes, C M R ^b  

^a ERASMUS UNIVERSITY ROTTERDAM   (Netherlands)

^b RADBOUD UNIVERSITY MEDICAL CENTER   (Netherlands)

^c ERASMUS MEDICAL CENTER   (Netherlands)

Author keywords

Class switch; DNA repair; Immunodeficiency; Immunoglobulins; Nijmegen Breakage synrdrome

Indexed keywords

CELL DNA; GENE PRODUCT; IMMUNOGLOBULIN A; IMMUNOGLOBULIN G; IMMUNOGLOBULIN M; NIBRIN; UNCLASSIFIED DRUG;

APOPTOSIS; ARTICLE; CELL CYCLE; CHROMOSOME DAMAGE; CONTROLLED STUDY; DNA REPAIR; GENE MUTATION; HOMOZYGOSITY; HUMAN; HUMAN CELL; IMMUNE DEFICIENCY; IMMUNOGLOBULIN BLOOD LEVEL; IMMUNOGLOBULIN GENE; MAJOR CLINICAL STUDY; MICROSATELLITE INSTABILITY; NIJMEGEN BREAKAGE SYNDROME; PREDICTION; PRIORITY JOURNAL; SIGNAL TRANSDUCTION;

AGE FACTORS; CD4-POSITIVE T-LYMPHOCYTES; CELL CYCLE PROTEINS; CHROMOSOME DISORDERS; DNA REPAIR; HUMANS; IGA DEFICIENCY; IGG DEFICIENCY; IMMUNOGLOBULIN CLASS SWITCHING; IMMUNOLOGIC DEFICIENCY SYNDROMES; NUCLEAR PROTEINS; SYNDROME;

EID: 0035208957     PISSN: 01988859     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0198-8859(01)00345-7     Document Type: Article

References (12)

1. A new chromosomal instability disorder: The Nijmegen breakage syndrome
2. Ataxia-telangiectasia and the Nijmegen breakage syndrome: Related disorders but genes apart
3. Nijmegen Breakage syndrome
4. Nibrin, a novel DNA double-strand break repair protein, is mutated in Nijmegen breakage syndrome
5. Disruption of mRad50 causes embryonic stem cell lethality, abnormal embryonic development, and sensitivity to ionizing radiation
6. The hMrel11/hRad 50 protein complex and Nijmegen breakage syndrome: Linkage of double-strand break repair to the cellular DNA damage response
7. Molecular mechanisms of DNA double strand break repair
8. An alternative mode of translation permits production of a variant NBS1 protein from the common Nijmegen breakage syndrome allele
9. Targeted disruption of the Nijmegen breakage syndrome gene NBS1 leads to early embryonic lethality in mice
10. Immunoglobulin class switch recombination: Will genetics provide new clues to mechanism?
11. Normal V(D)J recombination in cells from patients with Nijmegen breakage syndrome
12. Nijmegen Breakage syndrome: A progress report