Article
Characteristics of pulmonary arterial hypertension in affected carriers of a mutation located in the cytoplasmic tail of bone morphogenetic protein receptor type 2.
Chest - 1 May 2015
Girerd Barbara, Coulet Florence, Jaïs Xavier, Eyries Mélanie, Van Der Bruggen Cathelijne, De Man Frances, Houweling Arjan, Dorfmüller Peter, Savale Laurent, Sitbon Olivier, Vonk-Noordegraaf Anton, Soubrier Florent, Simonneau Gérald, Humbert Marc, Montani David
Abstract excerpt
BACKGROUND: Mutations in BMPR2 encoding bone morphogenetic protein receptor type 2 (BMPRII) is the main genetic risk factor for heritable pulmonary arterial hypertension (PAH). The suspected mechanism is considered to be a defect of BMP signaling. The BMPRII receptor exists in a short isoform wit...
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