Aggregation of the inactive form of human alpha-galactosidase in the endoplasmic reticulum

Biochemical and Biophysical Research Communications
S IshiiY Suzuki

Abstract

35S-Labeled mutant alpha-galactosidases (Q279E and R301Q) expressed in COS1 cells were detected as two forms (46-kDa protein and gel-top aggregate) on SDS-polyacrylamide gel electrophoresis under nonreducing conditions. The 46-kDa protein disappeared rapidly, but the aggregate decreased slowly. The accumulation of aggregate was observed in COS1 cells expressing either Q279E or R301Q on Western blot analysis. The aggregate was mainly recovered in the fraction extracted with 1% Triton X-100 and had no catalytic activity. The COS1 cells expressing Q279E were treated with 10 microgram/ml brefeldin A or 5 microM monensin. Treatment with brefeldin A caused a decrease in the alpha-galactosidase activity and an increase in the amount of aggregate, but the amount of aggregate did not change on monensin treatment.

Citations

Jul 10, 2012·The Journal of Biological Chemistry·Aleksandra SiekierskaFrederic Rousseau
Dec 17, 2003·Proceedings of the National Academy of Sciences of the United States of America·Junichiro MatsudaYoshiyuki Suzuki
Sep 27, 2007·The FEBS Journal·Jian-Qiang Fan, Satoshi Ishii
Oct 12, 2013·Biotechnology and Bioengineering·Simpson GregoireInchan Kwon
Sep 11, 2004·Protein Expression and Purification·Kayo YasudaJian-Qiang Fan
Jun 30, 2004·Trends in Endocrinology and Metabolism : TEM·Virginie BernierMichel Bouvier
Apr 18, 2006·Brain & Development·Hiroyuki IwasakiYoshiyuki Suzuki
Sep 23, 2016·Genetics in Medicine : Official Journal of the American College of Medical Genetics·Elfrida R BenjaminDavid J Lockhart
Jun 10, 2006·Journal of Inherited Metabolic Disease·Yoshiyuki Suzuki
Apr 9, 2013·The Korean Journal of Chemical Engineering·Simpson GregoireInchan Kwon
Dec 2, 2005·Journal of the American Society of Nephrology : JASN·James A Shayman
Mar 15, 2006·American Journal of Physiology. Cell Physiology·Gary Hin-Fai YamJürgen Roth
Sep 1, 2017·Future Medicinal Chemistry·Christoph Arenz
Oct 11, 2020·International Journal of Molecular Sciences·Hila BraunsteinMia Horowitz
Apr 11, 2003·Biochimica Et Biophysica Acta·Christopher J DanpureXiaoxuan Zhang
Oct 19, 2006·Biochimica Et Biophysica Acta·Tsutomu ArakawaKouhei Tsumoto
Mar 18, 2005·Journal of Medicinal Chemistry·Atsushi KatoNaoki Asano

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