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J Biol Chem, Vol. 274, Issue 39, 27392-27398, September 24, 1999
1(I) Chains
From the Department of Paediatrics, Orthopaedic Molecular Biology
Research Unit, University of Melbourne, Royal Children's Hospital,
Parkville, Victoria 3052, Australia
We have previously shown that type I procollagen
pro-
1(I) chains from an osteogenesis imperfecta patient (OI26) with
a frameshift mutation resulting in a truncated C-propeptide, have
impaired assembly, and are degraded by an endoplasmic
reticulum-associated pathway (Lamandé, S. R., Chessler,
S. D., Golub, S. B., Byers, P. H., Chan, D., Cole,
W. G., Sillence, D. O. and Bateman, J. F. (1995)
J. Biol. Chem. 270, 8642-8649). To further explore
the degradation of procollagen chains with mutant C-propeptides, mouse Mov13 cells, which produce no endogenous pro-1(I), were stably transfected with a pro-1(I) expression construct containing a frameshift mutation that predicts the synthesis of a protein 85 residues longer than normal. Despite high levels of mutant mRNA in
transfected Mov13 cells, only minute amounts of mutant pro-1(I) could be detected indicating that the majority of the mutant
pro-1(I) chains synthesized are targeted for rapid intracellular
degradation. Degradation was not prevented by brefeldin A, monensin, or
NH4Cl, agents that interfere with intracellular transport
or lysosomal function. However, mutant pro-1(I) chains in both
transfected Mov13 cells and OI26 cells were protected from proteolysis
by specific proteasome inhibitors. Together these data demonstrate for
the first time that procollagen chains containing C-propeptide mutations that impair assembly are degraded by the cytoplasmic proteasome complex, and that the previously identified endoplasmic reticulum-associated degradation of mutant pro-1(I) in OI26 is mediated by proteasomes.
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