Article

Transforming growth factor signalling: a common pathway in pulmonary arterial hypertension and systemic sclerosis.

Division of Respiratory, Critical Care and Occupational Pulmonary Medicine, Department of Internal Medicine, University of Utah, Salt Lake City, UT 84132, USA.
International journal of clinical practice. Supplement 08/2011; DOI:10.1111/j.1742-1241.2011.02726.x pp.35-43
Source: PubMed

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.

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Keywords

clinical condition characterised
 
conditions
 
epigenetic factors
 
genetic predisposition
 
HPAH
 
precapillary pulmonary hypertension
 
Pulmonary arterial hypertension
 
SSc
 
subcategorisation
 
systemic sclerosis
 
TGF-β superfamily signalling
 
various conditions