Functional analysis of bone morphogenetic protein type II receptor mutations underlying primary pulmonary hypertension.

@article{Rudarakanchana2002FunctionalAO,
  title={Functional analysis of bone morphogenetic protein type II receptor mutations underlying primary pulmonary hypertension.},
  author={N Rudarakanchana and Julia A Flanagan and Hailan Chen and Paul D Upton and Rajiv D Machado and Dipa Patel and Richard C Trembath and Nicholas W Morrell},
  journal={Human molecular genetics},
  year={2002},
  volume={11 13},
  pages={1517-25}
}
A wide range of mutations in the type II receptor for bone morphogenetic protein (BMPR-II) have been shown to underlie primary pulmonary hypertension. To determine the mechanism of altered BMPR-II function, we employed transient transfection studies in cell lines and primary cultures of pulmonary vascular smooth muscle cells using green fluorescent protein (GFP)-tagged wild-type and mutant BMPR2 constructs and confocal microscopy to localize receptors. Substitution of cysteine residues in the… CONTINUE READING
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