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

被引：12
|
作者：
Hatton, N. [1 ]
Frech, T. [2 ]
Smith, B. [1 ]
Sawitzke, A. [2 ]
Scholand, M. B. [1 ]
Markewitz, B. [1 ]
机构：
[1] Univ Utah, Div Resp Crit Care & Occupat Pulm Med, Salt Lake City, UT 84132 USA
[2] Univ Utah, Div Rheumatol, Dept Internal Med, Salt Lake City, UT 84132 USA
来源：
INTERNATIONAL JOURNAL OF CLINICAL PRACTICE | 2011年 / 65卷
关键词：
TGF-BETA RECEPTOR; GERMLINE MUTATIONS; RISK-FACTORS; SCLERODERMA; EXPRESSION; GENE; DISEASE; BMPR2; PATHOGENESIS; POLYMORPHISM;
D O I：
10.1111/j.1742-1241.2011.02726.x
中图分类号：
R5 [内科学];
学科分类号：
1002 ; 100201 ;
摘要：
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-beta 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-beta superfamily signalling in the pathogenesis of PAH and SSc.
引用
收藏
页码：35 / 43
页数：9
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