1xiw
Crystal structure of human CD3-e/d dimer in complex with a UCHT1 single-chain antibody fragmentCrystal structure of human CD3-e/d dimer in complex with a UCHT1 single-chain antibody fragment
Template:ABSTRACT PUBMED 15534202
DiseaseDisease
[CD3D_HUMAN] Defects in CD3D are a cause of severe combined immunodeficiency autosomal recessive T-cell-negative/B-cell-positive/NK-cell-positive (T(-)B(+)NK(+) SCID) [MIM:608971]. A form of severe combined immunodeficiency (SCID), a genetically and clinically heterogeneous group of rare congenital disorders characterized by impairment of both humoral and cell-mediated immunity, leukopenia, and low or absent antibody levels. Patients present in infancy recurrent, persistent infections by opportunistic organisms. The common characteristic of all types of SCID is absence of T-cell-mediated cellular immunity due to a defect in T-cell development.[1]
FunctionFunction
[CD3E_HUMAN] The CD3 complex mediates signal transduction. [CD3D_HUMAN] The CD3 complex mediates signal transduction.
About this StructureAbout this Structure
1xiw is a 8 chain structure with sequence from Homo sapiens and Mus musculus. Full crystallographic information is available from OCA.
ReferenceReference
- ↑ Dadi HK, Simon AJ, Roifman CM. Effect of CD3delta deficiency on maturation of alpha/beta and gamma/delta T-cell lineages in severe combined immunodeficiency. N Engl J Med. 2003 Nov 6;349(19):1821-8. PMID:14602880 doi:10.1056/NEJMoa031178