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Protein C Nagoya, an elongated mutant of protein C, is retained within the
endoplasmic reticulum and is associated with GRP78 and GRP94
A Katsumi, T Senda, Y Yamashita, T Yamazaki, M Hamaguchi, T Kojima, S Kobayashi and H Saito
First Department of Internal Medicine, Nagoya University School of
Medicine, Japan.
Protein C Nagoya, an elongated variant of the human protein C, is retained
and degraded within the cells in which it is produced (Yamamoto et al, J
Clin Invest 90:2439, 1992). To determine the subcellular localization of
the protein C Nagoya, the recombinant protein C bearing this mutation was
expressed in Chinese hamster ovary (CHO) cells. The mutant protein C was
not secreted from the cells and remained susceptible to
endo-beta-N-acetylglucosaminidase H (endo H). Immunoelectron microscopy
indicated that protein C Nagoya was retained in the endoplasmic reticulum
(ER), whereas wild-type protein C was observed in both the ER and the Golgi
apparatus. Metabolic radiolabeling with [35S] methionine in combination
with chemical cross- linking showed that the protein C Nagoya existed in
the ER as a complex with 78-kD glucose-regulated protein (GRP78) and 94-kD
glucose- regulated protein (GRP94). Because both GRP78 and GRP94 associate
to a far lesser degree with wild-type protein C than with protein C Nagoya,
our data suggest that both stress proteins function as molecular chaperones
and work in concert with the folding and assembly of protein C. These
findings extend our understanding the molecular pathogenesis of protein C
deficiency.
Volume 87,
Issue 10,
pp. 4164-4175,
05/15/1996
Copyright © 1996 by The American Society of Hematology
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