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J. Biol. Chem., Vol. 276, Issue 15, 11469-11472, April 13, 2001
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1 Latency-associated Peptide Cause Camurati-Engelmann Disease
Because of the Formation of a Constitutively Active Form of
TGF-1*
,
,,**
From the Transforming growth factor
(TGF)- Department of Biochemistry, Osaka University
Medical School, 2-2 Yamadaoka, Suita, Osaka 565-0871, the
§ Department of Human Genetics, Nagasaki University School
of Medicine, 12-4 Sakamoto, Nagasaki 852-8102, the ¶ Department of
Pediatrics and Public Health, Asahikawa Medical College, 2-1-1-1 Midorigaokahigashi, Asahikawa, Hokkaido 078-8510, and the
Department of Medical Genetics and Division of Clinical and
Molecular Genetics, Shinshu University School of Medicine, 3-1-1 Asahi,
Matsumoto, Nagano 390-8621, Japan
1 is secreted as a latent form, which consists of its mature
form and a latency-associated peptide (1-LAP) in either the presence
or the absence of additional latent TGF-1-binding protein. We
recently reported that three different missense mutations (R218H,
R218C, and C225R) of 1-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 1-LAP and TGF-1 and
the subsequent release of the mature TGF-1. Furthermore, the cell
growth of fibroblasts from a CED patient and mutant gene-transfected
fibroblasts was suppressed via TGF-1. The growth suppression
observed was attenuated by neutralizing antibody to TGF-1 or by
treatment of dexamethasone. On the other hand, the proliferation of
human osteoblastic MG-63 cells was accelerated by coculture with CED
fibroblasts. These data suggest that the domain-specific
mutations of 1-LAP result in a more facile activation of TGF-1,
thus causing CED.
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