1d4w: Difference between revisions
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{{STRUCTURE_1d4w| PDB=1d4w | SCENE= }} | {{STRUCTURE_1d4w| PDB=1d4w | SCENE= }} | ||
===CRYSTAL STRUCTURE OF THE XLP PROTEIN SAP IN COMPLEX WITH SLAM PHOSPHOPEPTIDE=== | |||
{{ABSTRACT_PUBMED_10549287}} | |||
=== | ==Disease== | ||
[[http://www.uniprot.org/uniprot/SH21A_HUMAN SH21A_HUMAN]] Defects in SH2D1A are a cause of lymphoproliferative syndrome X-linked type 1 (XLP1) [MIM:[http://omim.org/entry/308240 308240]]; also known as X-linked lymphoproliferative disease (XLPD) or Duncan disease. XLP is a rare immunodeficiency characterized by extreme susceptibility to infection with Epstein-Barr virus (EBV). Symptoms include severe or fatal mononucleosis, acquired hypogammaglobulinemia, pancytopenia and malignant lymphoma.<ref>PMID:11477068</ref><ref>PMID:9771704</ref><ref>PMID:11823424</ref><ref>PMID:10598819</ref><ref>PMID:11049992</ref><ref>PMID:11034354</ref><ref>PMID:11493483</ref><ref>PMID:14674764</ref><ref>PMID:15841490</ref><ref>PMID:16720617</ref> | |||
==Function== | |||
[[http://www.uniprot.org/uniprot/SH21A_HUMAN SH21A_HUMAN]] Inhibitor of the SLAM self-association. Acts by blocking recruitment of the SH2-domain-containing signal-transduction molecule SHP-2 to a docking site in the SLAM cytoplasmic region. Mediates interaction between FYN and SLAMF1. May also regulate the activity of the neurotrophin receptors NTRK1, NTRK2 and NTRK3. [[http://www.uniprot.org/uniprot/SLAF1_HUMAN SLAF1_HUMAN]] High-affinity self-ligand important in bidirectional T-cell to B-cell stimulation. SLAM-induced signal-transduction events in T-lymphocytes are different from those in B-cells. Two modes of SLAM signaling are likely to exist: one in which the inhibitor SH2D1A acts as a negative regulator and another in which protein-tyrosine phosphatase 2C (PTPN11)-dependent signal transduction operates. | |||
==About this Structure== | ==About this Structure== | ||
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==Reference== | ==Reference== | ||
<ref group="xtra">PMID:010549287</ref><references group="xtra"/> | <ref group="xtra">PMID:010549287</ref><references group="xtra"/><references/> | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Eck, M J.]] | [[Category: Eck, M J.]] | ||