Protein S Deficiency and The Mechanism of Protein S in Hemostasis and Atherosclerosis
Authors
Nur Feby Febiana Agistany , Ananda Karunia Ramadhan , Dira Kurnia Rizki , Raditya Bayu Farizil Akhyar , Ilsa HunaifiDOI:
10.29303/jbt.v23i4.5565Published:
2023-09-05Issue:
Vol. 23 No. 4 (2023): October - DecemberKeywords:
Atherosclerosis, coagulation, hemostasis, protein S Defficiency.Articles
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Abstract
Protein S (PS) is Vitamin K dependant protein which plays a role in regulating the blood clotting process in the body. Protein S deficiency is explained as a condition characterized by reduced levels of protein S in the serum so that it makes the blood clot too easily. The purpose of this literature review is to give an update of protein S defficiency and the mechanism of protein s in hemostasis and atherosclerosis based on publish literature reference from various database. Protein S plays important role in hemostasis by regulating blood cloth process in the initiation phase of the extrinsic pathway as a TFPI cofactor or in the propagation phase, namely as an APC cofactor and independent activity of TFPIα and APC. Protein S deficiency is known to be associated with the occurrence of atherosclerosis via the Tyro3-Axl-Mer (TAM) receptor. The main clinical manifestations of the majority of patients with heterozygous mutations in the protein S (PROS1) gene are deep vein thrombosis (DVT) and pulmonary embolism that precipitate by VTE. Administration of anticoagulant drugs such as heparin in the form of low-molecular-weight heparin (LMWH) or unfractionated heparin, vitamin K antagonists (VKA), namely warfarin, or direct oral anticoagulants (DOACs) can be selected as a management of protein S deficiency with VTE. Protein S has a central role in the coagulation prosses and its deficiency associated with atherosclerotic conditions.
References
Alshaikh, N. (2022). Protein S: a Central Regulator of Blood Coagulation. Clinical Laboratory, 68(08/2022), 1535–1542. Retrieved from https://doi.org/10.7754/Clin.Lab.2021.211010
Anderson, J. A. M., & Weitz, J. I. (2010). Hypercoagulable states. Clinics in Chest Medicine, 31(4), 659–673. Retrieved from https://doi.org/10.1016/j.ccm.2010.07.004
Ashish Gupta, Aung M. Tun, Kush Gupta, & Faiz Tuma. (2022). Protein S Deficiency. Retrieved 7 May 2023 from https://www.ncbi.nlm.nih.gov/books/NBK544344/
Chiasakul, T., De Jesus, E., Tong, J., Chen, Y., Crowther, M., Garcia, D., & Cuker, A. (2019). Inherited Thrombophilia and the Risk of Arterial Ischemic Stroke: A Systematic Review and Meta-Analysis. Journal of the American Heart Association, 8(19). Retrieved from https://doi.org/10.1161/JAHA.119.012877
Dahlbäck, B. (2018). Vitamin K–Dependent Protein S: Beyond the Protein C Pathway. Seminars in Thrombosis and Hemostasis, 44(02), 176–184. Retrieved from https://doi.org/10.1055/s-0037-1604092
Finsterer, J. (2023). Hereditary Protein S Deficiency and Activated Protein C Resistance Manifesting With Recurrent Thrombosis and Stroke. Cureus. Retrieved from https://doi.org/10.7759/cureus.34012
Gierula, M., & Ahnström, J. (2020). Anticoagulant protein S—New insights on interactions and functions. Journal of Thrombosis and Haemostasis, 18(11), 2801–2811. Retrieved from https://doi.org/10.1111/jth.15025
Gierula, M., Salles-Crawley, I. I., Santamaria, S., Teraz-Orosz, A., Crawley, J. T. B., Lane, D. A., & Ahnström, J. (2019). The roles of factor Va and protein S in formation of the activated protein C/protein S/factor Va inactivation complex. Journal of Thrombosis and Haemostasis, 17(12), 2056–2068. Retrieved from https://doi.org/10.1111/jth.14594
Hackeng, T. M., & Rosing, J. (2009). Protein s as cofactor for TFPI. Arteriosclerosis, Thrombosis, and Vascular Biology, 29(12), 2015–2020. Retrieved from https://doi.org/10.1161/ATVBAHA.108.177436
Heeb, M. J. (2008). Role of the PROS1 gene in thrombosis: lessons and controversies. NIH Public Access, 1(1), 9–12. Retrieved from https://doi.org/10.1586/17474086.1.1.9.Role
Klostermeier, U. C., Limperger, V., Kenet, G., Kurnik, K., Gelas, M. A., Finckh, U., & Nowak-Göttl, U. (2015). Role of protein S deficiency in children with venous thromboembolism. Thrombosis and Haemostasis, 113(02), 426–433. Retrieved from https://doi.org/10.1160/th14-06-0533
Libby, P., Buring, J. E., Badimon, L., Hansson, G. K., Deanfield, J., Bittencourt, M. S., & Lewis, E. F. (2019). Atherosclerosis. Nature Reviews Disease Primers, 5(1), 56. Retrieved from https://doi.org/10.1038/s41572-019-0106-z
Majumder, R., & Nguyen, T. (2021). Protein S: function, regulation, and clinical perspectives. Current Opinion in Hematology, 28(5), 339–344. Retrieved from https://doi.org/10.1097/MOH.0000000000000663
Meer, J. H. M. van der, Poll, T. van der, & Veer, C. van ’t. (2014). TAM receptors, Gas6, and protein S: Roles in inflammation and hemostasis. Blood, 123(16), 2460–2469. Retrieved from https://doi.org/10.1182/blood-2013-09-528752
Muchti, J. E., Anwar, Y., & Aman, A. K. (2019). Levels of protein C, protein S, and anti-thrombin III in acute ischemic stroke patients at Haj Adam Malik Hospital, Medan. Bali Medical Journal, 8(2), 460–464. Retrieved from https://doi.org/10.15562/bmj.v8i2.1378
Ohashi, I., Wada, S., Yoshino, F., Kuwashiro, T., Matsumoto, S., Hotta, T., & Yasaka, M. (2020). Ischemic Stroke with Protein S Deficiency Treated by Apixaban. Journal of Stroke and Cerebrovascular Diseases, 29(4), 104608. Retrieved from https://doi.org/10.1016/j.jstrokecerebrovasdis.2019.104608
Pilli, V., Plautz, W., & Majumder, R. (2016). The Journey of Protein S from an Anticoagulant to a Signaling Molecule. JSM Biochem Mol Biol, 3(1), 139–148. Retrieved from https://doi.org/10.1053/j.gastro.2016.08.014.CagY
Pintao, M. C., Ribeiro, D. D., Bezemer, I. D., Garcia, A. A., de Visser, M. C. H., Doggen, C. J. M., & Rosendaal, F. R. (2013). Protein S levels and the risk of venous thrombosis: results from the MEGA case-control study. Blood, 122(18), 3210–9. Retrieved from https://doi.org/10.1182/blood-2013-04-499335
Roshal, M., & Reyes Gil, M. (2019). Protein S Deficiency Evaluation. In Transfusion Medicine and Hemostasis (pp. 893–897). Elsevier. Retrieved from https://doi.org/10.1016/B978-0-12-813726-0.00151-3
Somajo, S., Ahnström, J., Fernandez-Recio, J., Gierula, M., Villoutreix, B. O., & Dahlbäck, B. (2015). Amino acid residues in the laminin G domains of protein S involved in tissue factor pathway inhibitor interaction. Thrombosis and Haemostasis, 113(5), 976–87. Retrieved from https://doi.org/10.1160/TH14-09-0803
Suleiman, L., Négrier, C., & Boukerche, H. (2013). Protein S: A multifunctional anticoagulant vitamin K-dependent protein at the crossroads of coagulation, inflammation, angiogenesis, and cancer. Critical Reviews in Oncology/Hematology, 88(3), 637–654. Retrieved from https://doi.org/10.1016/j.critrevonc.2013.07.004
Tàssies, D., & García De Frutos, P. (2014). Protein S deficiency and novel oral anticoagulants: An intriguing case. Thrombosis Research. Elsevier Ltd. Retrieved from https://doi.org/10.1016/j.thromres.2014.03.008
van der Meer, J. H. M., van der Poll, T., & van ‘t Veer, C. (2014). TAM receptors, Gas6, and protein S: roles in inflammation and hemostasis. Blood, 123(16), 2460–2469. Retrieved from https://doi.org/10.1182/blood-2013-09-528752
Wang, Z. H., Zhao, Z. J., Xu, K., Sun, G. B., Song, L., Yin, H. X., & Chen, X. Q. (2015). Hereditary protein S deficiency leads to ischemic stroke. Molecular Medicine Reports, 12(3), 3279–3284. Retrieved from https://doi.org/10.3892/mmr.2015.3793
Wolf, D., & Ley, K. (2019). Immunity and Inflammation in Atherosclerosis. Circulation Research, 124(2), 315–327. Retrieved from https://doi.org/10.1161/CIRCRESAHA.118.313591
Wypasek, E., & Undas, A. (2013). Protein C and protein S deficiency - practical diagnostic issues. Advances in Clinical and Experimental Medicine : Official Organ Wroclaw Medical University, 22(4), 459–67. Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/23986205
Wypasek, E., Karpinski, M., Alhenc-Gelas, M., & Undas, A. (2017). Venous thromboembolism associated with protein S deficiency due to Arg 451 ∗ mutation in PROS1 gene: a case report and a literature review. Journal of Genetics, 96(6), 1047–1051. Retrieved from https://doi.org/10.1007/s12041-017-0865-9
Yamazaki, H., Yagi, S., Torii, Y., Amano, R., Oomichi, Y., Sangawa, T., & Sata, M. (2018). Edoxaban improves acute venous thromboembolism while preserving protein C and protein S levels. Journal of Cardiology, 71(3), 305–309. Retrieved from https://doi.org/10.1016/j.jjcc.2017.09.009
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