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J Biol Chem, Vol. 274, Issue 13, 8933-8940, March 26, 1999
From the To investigate the processing and general
properties of the fibrillin-1 carboxyl-terminal domain, three protein
expression constructs have been developed as follows: one without the
domain, one with the domain, and one with a mutation near the putative proteolytic processing site. The constructs have been expressed in two
eukaryotic model systems, baculoviral and CHO-K1. Post-translational modifications that normally occur in fibrillin-1, including
glycosylation, signal peptide cleavage, and carboxyl-terminal
processing, occur in the three constructs in both cell systems.
Amino-terminal sequencing of secreted protein revealed leader sequence
processing at two sites, a primary site between Gly-24/Ala-25 and a
secondary site of Ala-27/Asn-28. Processing of the carboxyl-terminal
domain could be observed by migration differences in SDS-polyacrylamide
gel electrophoresis and was evident in both mammalian and insect cells. Immunological identification by Western blotting confirmed the loss of
the expected region. The failure of both cell systems to process the
mutant construct shows that the multi-basic sequence is the site of
proteolytic processing. Cleavage of the fibrillin-1 carboxyl-terminal
domain occurred intracellularly in CHO-K1 cells in an early secretory
pathway compartment as demonstrated by studies with secretion blocking
agents. This finding, taken with the multi-basic nature of the
cleavage site and observed calcium sensitivity of cleavage,
suggests that the processing enzyme is a secretory pathway resident
furin-like protease.
Processing of the Fibrillin-1 Carboxyl-terminal Domain
,,,
Department of Cell Biology and Physiology,
Washington University School of Medicine, St. Louis, Missouri 63110 and the § Department of Internal Medicine, University of
Texas-Houston Medical School, Houston, Texas 77030
Copyright © 1999 by The American Society for Biochemistry and Molecular Biology, Inc.
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