Update on the pathogenesis of hyper IgM syndrome
10.3760/cma.j.issn.1673-4408.2013.01.003
- VernacularTitle:高IgM综合征发病机制研究进展
- Author:
Wenjing TANG
- Publication Type:Journal Article
- Keywords:
Hyper IgM syndrome;
Class switch recombination defect;
Somatic hypermutation
- From:
International Journal of Pediatrics
2013;(1):10-13
- CountryChina
- Language:Chinese
-
Abstract:
The hyper immunoglobulin M syndromes(HIGM) are a heterogeneous group of genetic disorders resulting in defects of immunoglobulin class switch recombination,with or without defects of somatic hypermutation.They can be classified as defects of signalling through CD40 causing combined immunodeficiency,or intrinsic defects in B cells of the mechanism of class switch recombination resulting in a pure humoral immunodeficiency.This review summarizes the molecular pathogenesis of HIGM.
