Protein S
 | This article includes a list of general references, but it lacks sufficient corresponding inline citations. (August 2013) |
| PROS1 | |||
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Gene ontology | |||
| Molecular function | |||
| Cellular component | |||
| Biological process |
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Ensembl | |||||||||
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| RefSeq (protein) | |||||||||
| Location (UCSC) | Chr 3: 93.87 – 93.98 Mb | Chr 16: 62.67 – 62.75 Mb | |||||||
| PubMed search | [3] | [4] | |||||||
| View/Edit Human | View/Edit Mouse |
Protein S (also known as PROS) is a vitamin K-dependent plasma glycoprotein synthesized in the liver. In the circulation, Protein S exists in two forms: a free form and a complex form bound to complement protein C4b-binding protein (C4BP). In humans, protein S is encoded by the PROS1 gene.[5][6] Protein S plays a role in coagulation.
History
Protein S is named for Seattle, Washington, where it was originally discovered and purified[7] by Earl Davie's group in 1977.[8]
Structure
Protein S is partly
Additionally, protein S has a peptide between the Gla domain and the EGF-like domain, that is cleaved by
disulfide bond. However, protein S loses its function as an APC cofactor following either this cleavage or binding C4BP.[11]
Function
The best characterized function of Protein S is its role in the anti coagulation pathway, where it functions as a cofactor to Protein C in the inactivation of Factors Va and VIIIa. Only the free form has cofactor activity.[12]
Protein S binds to negatively charged
macrophages. Protein S binds to the negatively charged phospholipids and functions as a bridge between the apoptotic cell and the phagocyte. This bridging expedites phagocytosis and allows the cell to be removed without giving rise to inflammation
or other signs of tissue damage.
Protein S does not bind to the nascent complement complex C5,6,7 to prevents it from inserting into a membrane. This is a different complement protein S AKA vitronectin made by the VTN gene, not to be confused with the coagulation protein S made by the PROS gene which this wiki page concerns.
Pathology
Mutations in the PROS1 gene can lead to Protein S deficiency which is a rare blood disorder which can lead to an increased risk of thrombosis.[13][14]
Interactions
Protein S has been shown to interact with Factor V.[15][16]
See also
References
- ^ a b c GRCh38: Ensembl release 89: ENSG00000184500 – Ensembl, May 2017
- ^ a b c GRCm38: Ensembl release 89: ENSMUSG00000022912 – Ensembl, May 2017
- ^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
- ^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
- PMID 2944113.
- S2CID 31236887.
- ^ "Protein S deficiency". UpToDate. Retrieved May 10, 2017.
- ^ Kaushansky K, Lichtman M, Prchal J, Levi M, Press O, Burns L, Caligiuri M (2015). Williams Hematology. McGraw-Hill. p. 1926.
- PMID 10200912.
- PMID 1778174.
- PMID 2940598.
- S2CID 11522770.
- S2CID 705661.
- S2CID 17274778.
- S2CID 45995946.
- PMID 8428962.
Further reading
- Dahlbäck B (1991). "Protein S and C4b-binding protein: components involved in the regulation of the protein C anticoagulant system". Thromb. Haemost. 66 (1): 49–61. S2CID 24929072.
- Witt I (2002). "Molekularbiologische Grundlagen und Diagnostik der hereditären Defekte von Antithrombin III, Protein C und Protein S" [Molecular biological basis and diagnosis of hereditary defect of antithrombin III, protein c and protein S]. Hamostaseologie (in German). 22 (2): 57–66. S2CID 58077740.
- Rezende SM, Simmonds RE, Lane DA (2004). "Coagulation, inflammation, and apoptosis: different roles for protein S and the protein S-C4b binding protein complex". Blood. 103 (4): 1192–201. S2CID 133028.
- Dahlbäck B (2007). "The tale of protein S and C4b-binding protein, a story of affection". Thromb. Haemost. 98 (1): 90–6. S2CID 18823655.
- García de Frutos P, Fuentes-Prior P, Hurtado B, Sala N (2007). "Molecular basis of protein S deficiency". Thromb. Haemost. 98 (3): 543–56. S2CID 17274778.
- Maillard C, Berruyer M, Serre CM, et al. (1992). "Protein-S, a vitamin K-dependent protein, is a bone matrix component synthesized and secreted by osteoblasts". Endocrinology. 130 (3): 1599–604. PMID 1531628.
- Griffin JH, Gruber A, Fernández JA (1992). "Reevaluation of total, free, and bound protein S and C4b-binding protein levels in plasma anticoagulated with citrate or hirudin". Blood. 79 (12): 3203–11. PMID 1534488.)
{{cite journal}}: CS1 maint: DOI inactive as of April 2024 (link - Guglielmone HA, Vides MA (1992). "A novel functional assay of protein C in human plasma and its comparison with amidolytic and anticoagulant assays". Thromb. Haemost. 67 (1): 46–9. S2CID 27769717.
- Bertina RM, Ploos van Amstel HK, van Wijngaarden A, et al. (1990). "Heerlen polymorphism of protein S, an immunologic polymorphism due to dimorphism of residue 460". Blood. 76 (3): 538–48. PMID 2143091.
- Schmidel DK, Tatro AV, Phelps LG, et al. (1991). "Organization of the human protein S genes". Biochemistry. 29 (34): 7845–52. PMID 2148110.
- Ploos van Amstel HK, Reitsma PH, van der Logt CP, Bertina RM (1991). "Intron-exon organization of the active human protein S gene PS alpha and its pseudogene PS beta: duplication and silencing during primate evolution". Biochemistry. 29 (34): 7853–61. PMID 2148111.
- Allaart CF, Aronson DC, Ruys T, et al. (1991). "Hereditary protein S deficiency in young adults with arterial occlusive disease". Thromb. Haemost. 64 (2): 206–10. PMID 2148653.
- Ohlin AK, Landes G, Bourdon P, et al. (1989). "Beta-hydroxyaspartic acid in the first epidermal growth factor-like domain of protein C. Its role in Ca2+ binding and biological activity". J. Biol. Chem. 263 (35): 19240–8. PMID 2461936.
- Schwarz HP, Heeb MJ, Lottenberg R, et al. (1989). "Familial protein S deficiency with a variant protein S molecule in plasma and platelets". Blood. 74 (1): 213–21. PMID 2526663.
- Ploos van Amstel HK, van der Zanden AL, Reitsma PH, Bertina RM (1987). "Human protein S cDNA encodes Phe-16 and Tyr 222 in consensus sequences for the post-translational processing". FEBS Lett. 222 (1): 186–90. S2CID 46365357.
- Dahlbäck B, Lundwall A, Stenflo J (1986). "Primary structure of bovine vitamin K-dependent protein S". Proc. Natl. Acad. Sci. U.S.A. 83 (12): 4199–203. PMID 2940598.
- Lundwall A, Dackowski W, Cohen E, et al. (1986). "Isolation and sequence of the cDNA for human protein S, a regulator of blood coagulation". Proc. Natl. Acad. Sci. U.S.A. 83 (18): 6716–20. PMID 2944113.
- Engesser L, Broekmans AW, Briët E, et al. (1987). "Hereditary protein S deficiency: clinical manifestations". Ann. Intern. Med. 106 (5): 677–82. PMID 2952034.
- Watkins PC, Eddy R, Fukushima Y, et al. (1988). "The gene for protein S maps near the centromere of human chromosome 3". Blood. 71 (1): 238–41. PMID 2961379.
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