The present invention relates to an isolated BMP, (bone morphogonic protein) receptor kinase protein or soluble fragment thereof, a DNA sequence coding for the BMP receptor kinase protein or the soluble fragment thereof, a recombinant expression vector comprising the DNA sequence, a host cell comprising the recombinant expression vector, and a method of expressing the BMP receptor kinase protein or soluble fragment thereof.
This invention provides a method of detecting whether a subject is either predisposed to or afflicted with a pulmonary disease which comprises (1) obtaining a suitable sample from the subject; (2) detecting in the sample a bone morphogenetic protein receptor-II mutation which is not present in wildtype bone morphogenetic protein receptor-II, wherein the presence of a mutation indicates that the subject is predisposed to or afflicted with the pulmonary disease. In one embodiment, the pulmonary disease is Familial Primary Pulmonary Hypertension.