5IFH

Crystal structure of the BCR Fab fragment from subset #2 case P11475

5IFH の概要

• エントリーDOI: 10.2210/pdb5ifh/pdb
• 分子名称: Heavy chain of the Fab fragment from BCR derived from the P11475 CLL clone, Light chain of the Fab fragment from BCR derived from the P11475 CLL clone (3 entities in total)
• 機能のキーワード: immunoglobulin fold, b cell receptor, chronic lymphocytic leukemia, immune system
• 由来する生物種: Homo sapiens (Human); 詳細
• タンパク質・核酸の鎖数: 2
• 化学式量合計: 46976.19

構造登録者

Minici, C.,Degano, M. (登録日: 2016-02-26, 公開日: 2017-03-08, 最終更新日: 2024-01-10)

主引用文献

Minici, C.,Gounari, M.,Ubelhart, R.,Scarfo, L.,Duhren-von Minden, M.,Schneider, D.,Tasdogan, A.,Alkhatib, A.,Agathangelidis, A.,Ntoufa, S.,Chiorazzi, N.,Jumaa, H.,Stamatopoulos, K.,Ghia, P.,Degano, M.
Distinct homotypic B-cell receptor interactions shape the outcome of chronic lymphocytic leukaemia.
Nat Commun, 8:15746-15746, 2017

PubMed Abstract: Cell-autonomous B-cell receptor (BcR)-mediated signalling is a hallmark feature of the neoplastic B lymphocytes in chronic lymphocytic leukaemia (CLL). Here we elucidate the structural basis of autonomous activation of CLL B cells, showing that BcR immunoglobulins initiate intracellular signalling through homotypic interactions between epitopes that are specific for each subgroup of patients with homogeneous clinicobiological profiles. The molecular details of the BcR-BcR interactions apparently dictate the clinical course of disease, with stronger affinities and longer half-lives in indolent cases, and weaker, short-lived contacts mediating the aggressive ones. The diversity of homotypic BcR contacts leading to cell-autonomous signalling reconciles the existence of a shared pathogenic mechanism with the biological and clinical heterogeneity of CLL and offers opportunities for innovative treatment strategies.

PubMed: 28598442
DOI: 10.1038/ncomms15746

実験手法

X-RAY DIFFRACTION (2.293 Å)