title: Role of endoglin in fibrosis and scleroderma.
abstract: Fibrosis plays a role in many pathological conditions, among which is the autoimmune disease systemic sclerosis (SSc). SSc is characterized by fibrosis in the skin and internal organs, but the etiology remains to be elucidated. Transforming growth factor-β (TGF-β) is a key player in the fibrotic process, also in SSc. TGF-β induces the production of several components of the extracellular matrix and induces differentiation of fibroblasts to myofibroblasts, which further worsens fibrosis. Although TGF-β has been extensively investigated in fibrosis, the roles of several components of its signaling pathway are still unknown. Endoglin is a coreceptor for TGF-β and is known to modulate TGF-β signaling. Therefore, endoglin could enhance the effects of TGF-β in fibrosis or act as an inhibitor. Multiple studies have been conducted that support either hypothesis. Elucidating the exact role of endoglin in TGF-β signaling during fibrosis is important in understanding the process of fibrosis and could lead to the development of better treatments.
authors.affiliation: Department of Molecular Cell Biology and Centre for Biomedical Genetics, Leiden University Medical Center, Postzone S-1-P, Leiden, The Netherlands.
authors.forename: Janita A
authors.initials: JA
authors.lastname: Maring
authors.forename: Maria
authors.initials: M
authors.lastname: Trojanowska
authors.forename: Peter
authors.initials: P
authors.lastname: ten Dijke
mesh_terms: D000818:Animals; D005355:Fibrosis; D006801:Humans; D008954:Models, Biological; D011956:Receptors, Cell Surface / Q000378:metabolism*; D012595:Scleroderma, Systemic / Q000378:metabolism* / Q000473:pathology*; D015398:Signal Transduction; D016212:Transforming Growth Factor beta / Q000378:metabolism
chemical_list: D011956:Receptors, Cell Surface; D016212:Transforming Growth Factor beta