TY - JOUR
T1 - Characterization of bone morphogenetic protein 4 receptor in fibrodysplasia ossificans progressiva
AU - Lanchoney, Thomas F.
AU - Olmsted, Elizabeth A.
AU - Shore, Eileen M.
AU - Gannon, Francis A.
AU - Rosen, Vicki
AU - Zasloff, Michael A.
AU - Kaplan, Frederick S.
N1 - Copyright:
Copyright 2021 Elsevier B.V., All rights reserved.
PY - 1998/1
Y1 - 1998/1
N2 - 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. Semiquantitative 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.
AB - 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. Semiquantitative 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.
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U2 - 10.1097/00003086-199801000-00007
DO - 10.1097/00003086-199801000-00007
M3 - Article
C2 - 9577408
AN - SCOPUS:0031984848
SN - 0009-921X
VL - 346
SP - 38
EP - 45
JO - Clinical Orthopaedics and Related Research
JF - Clinical Orthopaedics and Related Research
ER -