- ►annals.org [PDF] JH Newman, JA Phillips III, JE Loyd - Annals of internal medicine, 2008 - Am Coll Physicians Pulmonary arterial hypertension (PAH) occurs as an idiopathic dis- ease
(formerly called primary pulmonary hypertension) and as a consequence of other
illnesses. These illnesses include connective tissue diseases, portal ... Cited by 18 - Related articles - All 14 versions
[CITATION] HOW DO MUTATIONS IN TGF-ß FAMILY RECEPTORS LEAD TO PAH?
R Hamid, JD Cogan, LK Hedges, E Austin, JA … - Hum Mutat, 2009 - interscience.wiley.com Familial pulmonary arterial hypertension (FPAH) is a progressive, fatal disease
caused by mutations in the bone morphogenetic protein receptor type 2 gene
(BMPR2). FPAH is inherited as an autosomal dominant trait, and shows ... Cited by 4 - Related articles - All 2 versions
ED Austin, JE Loyd - Clinics in chest medicine, 2007 - Elsevier Pulmonary arterial hypertension (PAH) is an uncommon disorder of the pulmonary
vasculature characterized by remodeling of the smallest pulmonary arteries,
leading to a progressive increase in pulmonary vascular resistance. Various ... Cited by 23 - Related articles - All 20 versions
WKP Wong, JH Morse, JA Knowles - Gene, 2006 - Elsevier A variety of mutations in the bone morphogenetic protein receptor type 2 (BMPR2)
have been identified in patients with pulmonary arterial hypertension. In this
study, using our BMPR2 mutation database and BMPR-II protein sequences from ... Cited by 5 - Related articles - All 5 versions
H Beppu, H Lei, KD Bloch, E Li - Developmental Genetics - interscience.wiley.com Bone morphogenetic proteins (BMPs) regulate a wide range of cellular functions
that contribute to embryonic development from mesoderm formation to
organogenesis. BMP type II receptor (BMPR-II) transduces BMP signals by ... Cited by 7 - Related articles - All 5 versions
C Kakiuchi, M Ishiwata, S Nanko, N Ozaki, N … - Am J Med Genet B Neuropsychiatr Genet, 2007 - interscience.wiley.com It is also possible that your web browser is not configured or not able to
display style sheets. In this case, although the visual presentation will be
degraded, the site should continue to be functional. We recommend using the ... Cited by 7 - Related articles - All 2 versions
- ►anmco.it [PDF] EB Rosenzweig, JH Morse, JA Knowles, KK … - Journal of Heart and Lung Transplantation, 2008 - Elsevier Of 147 patients (69 adults, 78 children; 114 with IPAH, 33 with FPAH), 124 (84%)
were BMPR2 mutation-negative, and 23 (16%) were mutation-positive. BMPR2
mutation-positive patients were less likely to respond to acute vasodilator ... Cited by 9 - Related articles - All 3 versions
- ►atsjournals.org JD Cogan, MW Pauciulo, AP Batchman, MA … - American journal of respiratory and critical care …, 2006 - Am Thoracic Soc Measurements and Main Results: Eleven (37%) patients with FPAH had abnormally
sized RT-PCR products. Four of the 11 patients were found to have splice-site
mutations resulting in aberrant splicing, and exonic deletions/duplications ... Cited by 35 - Related articles - BL Direct - All 9 versions
- ►171.66.122.149 B Sztrymf, F Coulet, B Girerd, A Yaici, X … - American Journal of Respiratory and Critical Care …, 2008 - 171.66.122.149 Measurements and Main Results: Sixty-eight BMPR2 mutation carriers (28 familial
and 40 idiopathic PAH) were compared with 155 noncarriers (all displaying
idiopathic PAH). As compared with noncarriers, BMPR2 mutation carriers were ... Cited by 25 - Related articles - BL Direct - All 4 versions
