2hac | pdb_00002hac

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Template:STRUCTURE 2hac

Structure of Zeta-Zeta Transmembrane Dimer

Template:ABSTRACT PUBMED 17055436

Disease

[CD3Z_HUMAN] Defects in CD247 are the cause of immunodeficiency due to defect in CD3-zeta (CD3ZID) [MIM:610163]. An immunological deficiency characterized by T-cells impaired immune response to alloantigens, tetanus toxoid and mitogens.[1]

Function

[CD3Z_HUMAN] Probable role in assembly and expression of the TCR complex as well as signal transduction upon antigen triggering.

About this Structure

2hac is a 2 chain structure with sequence from Homo sapiens. Full experimental information is available from OCA.

Reference

  1. Call ME, Schnell JR, Xu C, Lutz RA, Chou JJ, Wucherpfennig KW. The structure of the zetazeta transmembrane dimer reveals features essential for its assembly with the T cell receptor. Cell. 2006 Oct 20;127(2):355-68. PMID:17055436 doi:10.1016/j.cell.2006.08.044
  1. ↑ Rieux-Laucat F, Hivroz C, Lim A, Mateo V, Pellier I, Selz F, Fischer A, Le Deist F. Inherited and somatic CD3zeta mutations in a patient with T-cell deficiency. N Engl J Med. 2006 May 4;354(18):1913-21. PMID:16672702 doi:354/18/1913

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