GeneID: 219

Description:
The encoded protein belongs to the thrombospondin (TSP) family of multi-domain and multi-functional calcium-binding extracelullar glycoproteins. TSP-1 was first identified as a protein secreted from the α-granules of activated platelets. It is expressed at the cell surface during tissue development and remodelling, and functions as a matrix-based cellular signaling molecule. It can interact with a wide array of extracellular matrix (ECM) proteins to regulate various biological processes, including cell adhesion, proliferation, migration and angiogenesis. It is an activator of transforming growth factor (TGF-β). It has been involved in vascular homeostasis, wound healing and immunity. It plays a role in sickle erythrocyte adhesion to endothelium, which contributes to microvascular occlusion. Polymorphisms in this gene associated with pulmonary hypertension in sickle cell disease.

Synonyms: TSP , THBS , TSP1 , TSP-1 , THBS-1

Comments:
N/A

Number of entries/variants: 1

1. Brittain HA, Eckman JR, Swerlick RA, Howard RJ, Wick TM, Thrombospondin from activated platelets promotes sickle erythrocyte adherence to human microvascular endothelium under physiologic flow: a potential role for platelet activation in sickle cell vaso-occlusion., Blood , 81(8), 2137-43, 1993 PubMed
2. Tan K, Lawler J, The interaction of Thrombospondins with extracellular matrix proteins., J Cell Commun Signal , 3(3), 177-87, 2009 PubMed
3. Lopez-Dee Z, Pidcock K, Gutierrez LS, Thrombospondin-1: multiple paths to inflammation., Mediators Inflamm. , 2011(0), 296069, 2011 PubMed
4. Jacob SA, Novelli EM, Isenberg JS, Garrett ME, Chu Y, Soldano K, Ataga KI, Telen MJ, Ashley-Koch A, Gladwin MT, Zhang Y, Kato GJ, Thrombospondin-1 gene polymorphism is associated with estimated pulmonary artery pressure in patients with sickle cell anemia., Am. J. Hematol. , 92(3), E31-E34, 2017 PubMed