BMPR2 Haploinsufficiency as the Inherited Molecular Mechanism for Primary Pulmonary Hypertension

Primary pulmonary hypertension (PPH) is a potentially lethal disorder, because the elevation of the pulmonary arterial pressure may result in right-heart failure. Histologically, the disorder is characterized by proliferation of pulmonary-artery smooth muscle and endothelial cells, by intimal hyperp...

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Hauptverfasser: Machado, Rajiv D, Pauciulo, Michael W., Thomson, Jennifer R., Lane, Kirk B., Morgan, Neil V., Wheeler, Lisa, Phillips III, John A., Newman, John, Williams, Denise, Galiè, Nazzareno, Manes, Alessandra, McNeil, Keith, Yacoub, Magdi, Mikhail, Ghada, Rogers, Paula, Corris, Paul, Humbert, Marc, Donnai, Dian, Martensson, Gunnar, Tranebjaerg, Lisbeth, Loyd, James E., Trembath, Richard C., Nichols, William C.
Format: Artikel
Sprache:English
Veröffentlicht: The American Society of Human Genetics 2001
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Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC1234937/
https://ncbi.nlm.nih.gov/pubmed/11115378
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https://ncbi.nlm.nih.gov/pmc/articles/PMC1234937/
https://ncbi.nlm.nih.gov/pubmed/11115378

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