Article
Functional interaction between BMPR-II and Tctex-1, a light chain of Dynein, is isoform-specific and disrupted by mutations underlying primary pulmonary hypertension.
Human molecular genetics - 15 Dec 2003
Machado Rajiv D, Rudarakanchana Nung, Atkinson Carl, Flanagan Julia A, Harrison Rachel, Morrell Nicholas W, Trembath Richard C
Abstract excerpt
Diverse heterozygous mutations of bone morphogenetic receptor type II (BMPR-II) underlie the inherited form of the vascular disorder primary pulmonary hypertension (PPH). As yet, the molecular detail of how such defects contribute to the pathogenesis of PPH remains unclear. BMPR-II is a member of the transforming growth factor-beta cell signalling superfamily. Ligand binding induces cell surface receptor complex...
Topics
- Bone Morphogenetic Protein Receptors, Type II
- Dyneins
- HeLa Cells
- Humans
- Hypertension, Pulmonary
- Lung
- Microtubule-Associated Proteins
- Models, Biological
- Mutation
- Nuclear Proteins
- Phosphorylation
- Plasmids
- Protein Isoforms