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{{STRUCTURE_4dom| PDB=4dom | SCENE= }} | |||
===Crystal Structure of the TIR-domain of Human Myeloid Differentiation Primary Response protein (MyD88)=== | |||
==Disease== | |||
[[http://www.uniprot.org/uniprot/MYD88_HUMAN MYD88_HUMAN]] Defects in MYD88 are the cause of MYD88 deficiency (MYD88D) [MIM:[http://omim.org/entry/612260 612260]]; also known as recurrent pyogenic bacterial infections due to MYD88 deficiency. Patients suffer from autosomal recessive, life-threatening, often recurrent pyogenic bacterial infections, including invasive pneumococcal disease, and die between 1 and 11 months of age. Surviving patients are otherwise healthy, with normal resistance to other microbes, and their clinical status improved with age.<ref>PMID:19506249</ref> <ref>PMID:18669862</ref> | |||
==Function== | |||
[[http://www.uniprot.org/uniprot/MYD88_HUMAN MYD88_HUMAN]] Adapter protein involved in the Toll-like receptor and IL-1 receptor signaling pathway in the innate immune response. Acts via IRAK1, IRAK2, IRF7 and TRAF6, leading to NF-kappa-B activation, cytokine secretion and the inflammatory response. Increases IL-8 transcription. Involved in IL-18-mediated signaling pathway. Activates IRF1 resulting in its rapid migration into the nucleus to mediate an efficient induction of IFN-beta, NOS2/INOS, and IL12A genes (By similarity).<ref>PMID:9013863</ref> <ref>PMID:15361868</ref> <ref>PMID:18292575</ref> <ref>PMID:19506249</ref> | |||
==About this Structure== | |||
[[4dom]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4DOM OCA]. | |||
==Reference== | |||
<references group="xtra"/><references/> | |||
[[Category: Homo sapiens]] | |||
[[Category: Jiang, J S.]] | |||
[[Category: Snyder, G A.]] | |||
[[Category: Xiao, T.]] | |||
[[Category: Adopter domain]] | |||
[[Category: Innate immune signaling]] | |||
[[Category: Lysine methylation]] | |||
[[Category: Myd88_human]] | |||
[[Category: Signaling protein]] | |||
[[Category: Tir-domain]] | |||
[[Category: Tirap/mal]] | |||
[[Category: Toll-like receptor]] |