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PROS1/Protein S  Protein, Antibody, ELISA Kit, cDNA Clone

PROS1/Protein S Related Area

PROS1/Protein S 関連経路

    PROS1/Protein S サマリー & タンパク質情報

    PROS1/Protein S 背景

    細胞内位置: Secreted.
    組織特異性: Plasma.
    翻訳後: The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains. {ECO:0000250}.
    疾患関連性: DISEASE: Thrombophilia due to protein S deficiency, autosomal dominant (THPH5) [MIM:612336]: A hemostatic disorder characterized by impaired regulation of blood coagulation and a tendency to recurrent venous thrombosis. Based on the plasma levels of total and free PROS1 as well as the serine protease-activated protein C cofactor activity, three types of THPH5 have been described: type I, characterized by reduced total and free PROS1 levels together with reduced anticoagulant activity; type III, in which only free PROS1 antigen and PROS1 activity levels are reduced; and the rare type II which is characterized by normal concentrations of both total and free PROS1 antigen, but low cofactor activity. {ECO:0000269|PubMed:10447256, ECO:0000269|PubMed:10613647, ECO:0000269|PubMed:10706858, ECO:0000269|PubMed:10790208, ECO:0000269|PubMed:11372770, ECO:0000269|PubMed:11776305, ECO:0000269|PubMed:11858485, ECO:0000269|PubMed:11927129, ECO:0000269|PubMed:12351389, ECO:0000269|PubMed:12632031, ECO:0000269|PubMed:15238143, ECO:0000269|PubMed:15712227, ECO:0000269|PubMed:7482398, ECO:0000269|PubMed:7545463, ECO:0000269|PubMed:7579449, ECO:0000269|PubMed:7803790, ECO:0000269|PubMed:8298131, ECO:0000269|PubMed:8639833, ECO:0000269|PubMed:8701404, ECO:0000269|PubMed:8765219, ECO:0000269|PubMed:8781426, ECO:0000269|PubMed:8943854, ECO:0000269|PubMed:8977443, ECO:0000269|PubMed:9031443, ECO:0000269|PubMed:9241758, ECO:0000269|Ref.15}. Note=The disease is caused by mutations affecting the gene represented in this entry.; DISEASE: Thrombophilia due to protein S deficiency, autosomal recessive (THPH6) [MIM:614514]: A very rare and severe hematologic disorder resulting in thrombosis and secondary hemorrhage usually beginning in early infancy. Some affected individuals develop neonatal purpura fulminans, multifocal thrombosis, or intracranial hemorrhage. {ECO:0000269|PubMed:20484936}. Note=The disease is caused by mutations affecting the gene represented in this entry.
    シーケンスの類似性: Contains 4 EGF-like domains. {ECO:0000255|PROSITE-ProRule:PRU00076}.; Contains 1 Gla (gamma-carboxy-glutamate) domain. {ECO:0000255|PROSITE-ProRule:PRU00463}.; Contains 2 laminin G-like domains. {ECO:0000255|PROSITE-ProRule:PRU00122}.
    General information above from UniProt

    PROS1, also known as protein S, is a vitamin K-dependent plasma protein that functions as a cofactor for the anticoagulant protease, activated protein C (APC) to inhibit blood coagulation. PROS1 has two isoforms: a free, functionally active form and an inactive form complexed with C4b-binding protein. Besides its anticoagulant function, PROS1 also acts as an agonist for the tyrosine kinase receptors Tyro3, Axl, and Mer. The endothelium expresses Tyro3, Axl, and Mer and produces protein S. The interaction of protein S with endothelial cells and particularly its effects on angiogenesis have not yet been analyzed.

    PROS1/Protein S 代替名

    Protein S, []
    PSA,PROS,PS21,PS22,PS23,PS24,PS25,THPH5,THPH6, [homo-sapiens]
    protein S (alpha),PROS1,PROS,PS21,PS22,PS23,PS24,PS25,PSA,THPH5,THPH6,protein Sa,vitamin K-dependent plasma protein S,vitamin K-dependent protein S, [human]
    protein S (alpha),Pros1,AW214361,vitamin K-dependent protein S, [mouse]
    AW214361, [mus-musculus]

    PROS1/Protein S 関連研究

  • Beauchamp NJ. et al., 2004, Br J Haematol. 125 (5): 647-54.
  • García de Frutos P. et al., 2007, Thromb Haemost. 98 (3): 543-56.
  • Rezende SM. et al., 2004, Blood. 103 (4): 1192-201.
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