title: Transforming growth factor signalling: a common pathway in pulmonary arterial hypertension and systemic sclerosis.
abstract: Pulmonary arterial hypertension (PAH) is a clinical condition characterised by the presence of precapillary pulmonary hypertension (PH). Included within the subcategorisation of PAH are heritable (HPAH) and PAH associated various conditions (APAH) including systemic sclerosis (SSc). The pathogenesis of HPAH and SSc has been linked to both a genetic predisposition and epigenetic factors. TGF-β superfamily signalling has also been implicated in the development of these conditions. In this review, we discuss the role of genetic predisposition, epigenetic factors along with dysregulation in TGF-β superfamily signalling in the pathogenesis of PAH and SSc.
authors: Division of Respiratory, Critical Care and Occupational Pulmonary Medicine, Department of Internal Medicine, University of Utah, Salt Lake City, UT 84132, USA. nathan.hatton@hsc.utah.edu
authors: N
authors: N
authors: Hatton
authors: T
authors: T
authors: Frech
authors: B
authors: B
authors: Smith
authors: A
authors: A
authors: Sawitzke
authors: M B
authors: MB
authors: Scholand
authors: B
authors: B
authors: Markewitz
mesh_terms: D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D020022:Genetic Predisposition to Disease / Q000453:epidemiology*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology / Q000235:genetics* / Q000503:physiopathology; D008297:Male; D015995:Prevalence; D011379:Prognosis; D018125:Receptors, Transforming Growth Factor beta / Q000235:genetics*; D018570:Risk Assessment; D012595:Scleroderma, Systemic / Q000453:epidemiology / Q000235:genetics* / Q000503:physiopathology; D015398:Signal Transduction / Q000235:genetics; D016019:Survival Analysis; D015291:Transforming Growth Factors / Q000235:genetics
chemical_list: D018125:Receptors, Transforming Growth Factor beta; D015291:Transforming Growth Factors