Pulmonary Arterial Hypertension: a Disease of Tethers, Snares and Snaps?
From: Department of Cell Biology and Anatomy, New York Medical College, Valhalla, NY 10595, USA. pravin_sehgal@nymc.edu
American journal of physiology. Heart and circulatory physiology
- Publish Date: Jul 2007
- ISSN: 0363-6135
- Volume: 293
- Issue: 1
- Pages: H77-85
- Medium: Print
- Language: English
- Citation (JAMA): Sehgal Pravin B, Mukhopadhyay Somshuvra, et al. Pulmonary Arterial Hypertension: a Disease of Tethers, Snares and Snaps?. Am. J. Physiol. Heart Circ. Physiol. Jul 2007;293:H77-85
Abstract
Histological and electron microscopic studies over the past four decades have highlighted “plump,” “enlarged” endothelial, smooth muscle, and fibroblastic cellular elements with increased endoplasmic reticulum, Golgi stacks, and vacuolation in pulmonary arterial lesions in human and in experimental (hypoxia and monocrotaline) pulmonary arterial hypertension. However, the contribution of disrupted intracellular membrane trafficking in the pathobiology of this disease has received insufficient attention. Recent studies suggest a pathogenetic role of the disruption of intracellular trafficking of vasorelevant proteins and cell-surface receptors in the development of this disease. The purpose of this essay is to highlight the molecular regulation of vesicular trafficking by membrane tethers, SNAREs and SNAPs, and to suggest how their dysfunction, directly and/or indirectly, might contribute to development of pulmonary arterial hypertension in experimental models and in humans, including that due to mutations in bone morphogenetic receptor type 2.
Mesh Headings (Keywords): Animals, Endothelium, Vascular, Humans, Hypertension, Pulmonary, Models, Cardiovascular, Pulmonary Artery, SNARE Proteins, Vesicular Transport Proteins
Check for Full Text / PubMed Unique Identifier (PMID): 17416597
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