title: Characterization of bone morphogenetic protein 4 receptor in fibrodysplasia ossificans progressiva.
abstract: Bone morphogenetic protein 4, a potent osteogenic morphogen, has been implicated in fibrodysplasia ossificans progressiva because it is uniquely overexpressed in lymphoblastoid cells and preosseous fibroproliferative lesional cells of patients with fibrodysplasia ossificans progressiva. Bone morphogenetic protein 4 signals through a heteromeric complex of serine/ threonine kinase receptors (type I and type II) on the surface of responding cells. Semi-quantitative competitive reverse transcription polymerase chain reaction was used to quantitate steady state levels of messenger ribonucleic acid expression for bone morphogenetic protein 4 and the bone morphogenetic protein receptors. These data confirmed the previous finding of elevated steady state levels of bone morphogenetic protein 4 messenger ribonucleic acid in lymphoblastoid cell lines of affected individuals in a family that exhibited autosomal dominant inheritance of fibrodysplasia ossificans progressiva. There were no differences in the steady state levels of messenger ribonucleic acid for either the Type I or Type II bone morphogenetic protein 4 receptors between affected and unaffected individuals in that same family. The presence of bone morphogenetic protein 4 receptor messenger ribonucleic acid in fibrodysplasia ossificans progressiva lesional tissue and unaffected muscle tissue and demonstrates the deregulation of bone morphogenetic protein 4 messenger ribonucleic acid in fibrodysplasia ossificans progressiva. These data support the hypothesis that the molecular basis of bone morphogenetic protein 4 signaling is abnormal in fibrodysplasia ossificans progressiva.
authors.affiliation: Department of Orthopaedic Surgery, University of Pennsylvania School of Medicine, Philadelphia 19104, USA.
authors.forename: T F
authors.initials: TF
authors.lastname: Lanchoney
authors.forename: E A
authors.initials: EA
authors.lastname: Olmsted
authors.forename: E M
authors.initials: EM
authors.lastname: Shore
authors.forename: F A
authors.initials: FA
authors.lastname: Gannon
authors.forename: V
authors.initials: V
authors.lastname: Rosen
authors.forename: M A
authors.initials: MA
authors.lastname: Zasloff
authors.forename: F S
authors.initials: FS
authors.lastname: Kaplan
mesh_terms: D055415:Bone Morphogenetic Protein 4; D052004:Bone Morphogenetic Protein Receptors; D019485:Bone Morphogenetic Proteins / Q000378:metabolism*; D002478:Cells, Cultured; D002648:Child; D005260:Female; D006801:Humans; D009221:Myositis Ossificans / Q000235:genetics / Q000378:metabolism*; D010375:Pedigree; D016133:Polymerase Chain Reaction; D012333:RNA, Messenger / Q000032:analysis; D011956:Receptors, Cell Surface / Q000378:metabolism*; D017978:Receptors, Growth Factor; D014158:Transcription, Genetic; D014407:Tumor Cells, Cultured
chemical_list: C525809:BMP4 protein, human; D055415:Bone Morphogenetic Protein 4; D019485:Bone Morphogenetic Proteins; D012333:RNA, Messenger; D011956:Receptors, Cell Surface; D017978:Receptors, Growth Factor; D052004:Bone Morphogenetic Protein Receptors