3kg5
Crystal structure of human Ig-beta homodimerCrystal structure of human Ig-beta homodimer
Template:ABSTRACT PUBMED 20696394
DiseaseDisease
[CD79B_HUMAN] Autosomal agammaglobulinemia. Defects in CD79B are the cause of agammaglobulinemia type 6 (AGM6) [MIM:612692]. It is a primary immunodeficiency characterized by profoundly low or absent serum antibodies and low or absent circulating B-cells due to an early block of B-cell development. Affected individuals develop severe infections in the first years of life.[1]
FunctionFunction
[CD79B_HUMAN] Required in cooperation with CD79A for initiation of the signal transduction cascade activated by the B-cell antigen receptor complex (BCR) which leads to internalization of the complex, trafficking to late endosomes and antigen presentation. Enhances phosphorylation of CD79A, possibly by recruiting kinases which phosphorylate CD79A or by recruiting proteins which bind to CD79A and protect it from dephosphorylation.[2] [3] [4]
About this StructureAbout this Structure
3kg5 is a 2 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
ReferenceReference
- ↑ Radaev S, Zou Z, Tolar P, Nguyen K, Nguyen A, Krueger PD, Stutzman N, Pierce S, Sun PD. Structural and functional studies of Igalphabeta and its assembly with the B cell antigen receptor. Structure. 2010 Aug 11;18(8):934-43. PMID:20696394 doi:10.1016/j.str.2010.04.019
- ↑ Dobbs AK, Yang T, Farmer D, Kager L, Parolini O, Conley ME. Cutting edge: a hypomorphic mutation in Igbeta (CD79b) in a patient with immunodeficiency and a leaky defect in B cell development. J Immunol. 2007 Aug 15;179(4):2055-9. PMID:17675462
- ↑ Luisiri P, Lee YJ, Eisfelder BJ, Clark MR. Cooperativity and segregation of function within the Ig-alpha/beta heterodimer of the B cell antigen receptor complex. J Biol Chem. 1996 Mar 1;271(9):5158-63. PMID:8617796
- ↑ Tseng J, Eisfelder BJ, Clark MR. B-cell antigen receptor-induced apoptosis requires both Ig alpha and Ig beta. Blood. 1997 Mar 1;89(5):1513-20. PMID:9057631
- ↑ Pelanda R, Braun U, Hobeika E, Nussenzweig MC, Reth M. B cell progenitors are arrested in maturation but have intact VDJ recombination in the absence of Ig-alpha and Ig-beta. J Immunol. 2002 Jul 15;169(2):865-72. PMID:12097390