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Titolo:
Bone morphogenetic proteins, genetics and the pathophysiology of primary pulmonary hypertension
Autore:
De Caestecker, M; Meyrick, B;
Indirizzi:
Vanderbilt Univ, Med Ctr, Dept Med, Nashville, TN 37232 USA Vanderbilt Univ Nashville TN USA 37232 Dept Med, Nashville, TN 37232 USA Vanderbilt Univ, Med Ctr, Dept Pathol, Nashville, TN 37232 USA Vanderbilt Univ Nashville TN USA 37232 pt Pathol, Nashville, TN 37232 USA
Titolo Testata:
RESPIRATORY RESEARCH
fascicolo: 4, volume: 2, anno: 2001,
pagine: 193 - 197
SICI:
1465-993X(2001)2:4<193:BMPGAT>2.0.ZU;2-F
Fonte:
ISI
Lingua:
ENG
Soggetto:
II RECEPTOR; GERMLINE MUTATIONS; PROLIFERATION; EXPRESSION; REGULATORS; COMPLEXES; EMBRYOS; TAK1;
Keywords:
bone morphogenetic protein type II receptor (BMPR-II) gene mutations; morphology; pulmonary arteries; transforming growth factor (TGF)-beta superfamily;
Tipo documento:
Editorial Material
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
33
Recensione:
Indirizzi per estratti:
Indirizzo: Meyrick, B Vanderbilt Univ, Med Ctr, Ctr Lung Res, Nashville, TN 37232 USAVanderbilt Univ Nashville TN USA 37232 Nashville, TN 37232 USA
Citazione:
M. De Caestecker e B. Meyrick, "Bone morphogenetic proteins, genetics and the pathophysiology of primary pulmonary hypertension", RESPIR RES, 2(4), 2001, pp. 193-197

Abstract

Several recent papers have shown that both familial primary pulmonary hypertension (FPPH) and sporadic primary pulmonary hypertension (PPH) may have a common etiology that is associated with the inheritance and/or spontaneous development of germline mutations in the bone morphogenetic protein receptor (BMPR) type II gene. Because BMPR-II is a ubiquitously expressed receptor for a family of secreted growth factors known as the bone morphogenetic proteins (BMPs), these findings suggest that BMPs play an important role inthe maintenance of normal pulmonary vascular physiology. In the present commentary we discuss the implications of these findings in the context of BMP receptor biology, and relate these data to the genetics and pulmonary pathophysiology of patients with PPH.

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Documento generato il 01/10/20 alle ore 16:16:33