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Papers In Press, published online ahead of print February 13, 2001
J. Biol. Chem, 10.1074/jbc.C000859200
Submitted on December 5, 2000
Revised on February 2, 2001
Accepted on February 12, 2001
Biochemistry, Osaka University Medical School, Suita, Osaka 565-0871
Corresponding Author: proftani{at}biochem.med.osaka-u.ac.jp
TGF-beta1 is secreted as a latent form which consists of its mature form and a latency-associated peptide (beta1-LAP) in either the presence or the absence of additional latent TGF-beta1-binding protein (LTBP). We recently reported that three different missense mutations (R218H, R218C and C225R) of beta1-LAP cause the Camurati-Engelmann disease (CED), an autosomal dominant disorder characterized by hyperosteosis and sclerosis of the diaphysis of the long bones. Pulse-chase experiments using fibroblasts from CED patients and expression experiments of the mutant genes in an insect cell system suggest that these mutations disrupt the association of beta1-LAP and TGF-beta1 and the subsequent release of the mature TGF-beta1. Furthermore, the cell growth of fibroblasts from a CED patient and mutant gene-transfected fibroblasts was suppressed via TGF-beta1. The growth suppression observed was attenuated by neutralizing antibody to TGF-beta1 or by treatment of dexamethasone. On the other hand, the proliferation of human osteoblastic MG-63 cells was accelerated by co-culture with CED fibroblasts. These data suggest that the domain specific mutations of beta1-LAP result in a more facile activation of TGF-beta1, thus causing CED.
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