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Cellular and molecular pathobiology of pulmonary arterial hypertension.

• Marc Humbert, Nicholas W Morrell, +8 authors Marlene Rabinovitch
• Journal of the American College of Cardiology
• 2004

Pulmonary arterial hypertension (PAH) has a multifactorial pathobiology. Vasoconstriction, remodeling of the pulmonary vessel wall, and thrombosis contribute to increased pulmonary vascular… (More)

Altered growth responses of pulmonary artery smooth muscle cells from patients with primary pulmonary hypertension to transforming growth factor-beta(1) and bone morphogenetic proteins.

• Nicholas W Morrell, Xiuwei Yang, +4 authors Richard C Trembath
• Circulation
• 2001

BACKGROUND Mutations in the type II receptor for bone morphogenetic protein (BMPR-II), a receptor member of the transforming growth factor-beta (TGF-beta) superfamily, underlie many cases of familial… (More)

Clinical and molecular genetic features of pulmonary hypertension in patients with hereditary hemorrhagic telangiectasia.

• Richard C Trembath, Jennifer R. Thomson, +14 authors Lamar Wheeler
• The New England journal of medicine
• 2001

BACKGROUND Most patients with familial primary pulmonary hypertension have defects in the gene for bone morphogenetic protein receptor II (BMPR2), a member of the transforming growth factor beta… (More)

Dysfunctional Smad signaling contributes to abnormal smooth muscle cell proliferation in familial pulmonary arterial hypertension.

• Xudong D Yang, L. -K. Long, +7 authors Nicholas W Morrell
• Circulation research
• 2005

Mutations in the bone morphogenetic protein type II receptor gene (BMPR2) are the major genetic cause of familial pulmonary arterial hypertension (FPAH). Although smooth muscle cell proliferation… (More)

Cellular and molecular basis of pulmonary arterial hypertension.

• Nicholas W Morrell, Serge Adnot, +9 authors Edward Kenneth Weir
• Journal of the American College of Cardiology
• 2009

Pulmonary arterial hypertension (PAH) is caused by functional and structural changes in the pulmonary vasculature, leading to increased pulmonary vascular resistance. The process of pulmonary… (More)

Primary pulmonary hypertension is associated with reduced pulmonary vascular expression of type II bone morphogenetic protein receptor.

• C Scott Atkinson, Selden Stewart, +4 authors Nicholas W Morrell
• Circulation
• 2002

BACKGROUND Mutations in the type II receptor for bone morphogenetic protein (BMPR-II), a receptor member of the transforming growth factor-beta (TGF-beta) superfamily, underlie many familial and… (More)

Dynamic changes in lung microRNA profiles during the development of pulmonary hypertension due to chronic hypoxia and monocrotaline.

• Paola Caruso, Margaret R. Maclean, +12 authors Andrew H. Baker
• Arteriosclerosis, thrombosis, and vascular…
• 2010

OBJECTIVE MicroRNAs (miRNAs) are small noncoding RNAs that have the capacity to control protein production through binding "seed" sequences within a target mRNA. Each miRNA is capable of potentially… (More)

Elevated levels of inflammatory cytokines predict survival in idiopathic and familial pulmonary arterial hypertension.

• Elaine Soon, Alan Holmes, +11 authors Nicholas W Morrell
• Circulation
• 2010

BACKGROUND Inflammation is a feature of pulmonary arterial hypertension (PAH), and increased circulating levels of cytokines are reported in patients with PAH. However, to date, no information exists… (More)

Molecular and functional analysis identifies ALK-1 as the predominant cause of pulmonary hypertension related to hereditary haemorrhagic telangiectasia.

• Rachel E Harrison, Julia A Flanagan, +14 authors Richard C Trembath
• Journal of medical genetics
• 2003

BACKGROUND Mutations of the transforming growth factor beta (TGFbeta) receptor components ENDOGLIN and ALK-1 cause the autosomal dominant vascular disorder hereditary haemorrhagic telangiectasia… (More)

A novel murine model of severe pulmonary arterial hypertension.

• Loredana Ciuclan, Olivier Bonneau, +10 authors Matthew T. Thomas
• American journal of respiratory and critical care…
• 2011

RATIONALE The complex pathologies associated with severe pulmonary arterial hypertension (PAH) in humans have been a challenge to reproduce in mice due to the subtle phenotype displayed to PAH… (More)

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