Relationship of Thrombospondin 1 to von Willebrand Factor and ADAMTS-13 in Sickle Cell Disease Patients of Arab Ethnicity

• Published in: Acta haematologica Vol. 144; no. 2; p. 182
• Main Authors: Al-Awadhi, Anwar, Adekile, Adekunle, Marouf, Rajaa
• Format: Journal Article
• Language: English
• Published: Switzerland 01.03.2021
• Subjects: ADAMTS13 Protein - analysis; ADAMTS13 Protein - metabolism; Adult; Anemia, Sickle Cell - ethnology; Anemia, Sickle Cell - genetics; Anemia, Sickle Cell - pathology; Arabs; Blood Cell Count; Case-Control Studies; Child; Female; Hemolysis; Humans; Linear Models; Male; Middle Aged; Thrombospondin 1 - analysis; Thrombospondin 1 - metabolism; von Willebrand Factor - analysis; von Willebrand Factor - metabolism; ADAMTS-13; Sickle cell disease; Thrombospondin 1; von Willebrand factor

Thrombospondin 1 (TSP-1) is a multifunctional glycoprotein secreted by platelets. In sickle cell disease (SCD), TSP-1 promotes red cell adhesion to the endothelium by binding to von Willebrand factor (vWF) and inhibiting its degradation by the protease ADAMTS-13. We investigated a possible correlati...