Suppressed disassembly of autolyzing p94/CAPN3 by N2A connectin/titin in a genetic reporter system

The Journal of Biological Chemistry
Yasuko OnoHiroyuki Sorimachi

Abstract

p94/calpain 3 is a skeletal muscle-specific member of the Ca(2+)-regulated cytosolic cysteine protease family, the calpains. Defective p94 protease activity originating from gene mutations causes a muscular dystrophy called calpainopathy, indicating the indispensability of p94 for muscle survival. Because of the existence of the p94-specific regions IS1 and IS2, p94 undergoes very rapid and exhaustive autolysis. To elucidate the physiological relevance of this unique activity, the autolytic profiles of p94 and the effect of the p94 binding protein, connectin/titin, on this process were investigated. In vitro analysis of p94 autolysis showed that autolysis in IS1 proceeds without immediate disassembly into fragments and that the newly identified cryptic autolytic site in IS2 is critical for disassembling autolyzed fragments. As a genetic system to assay p94 autolysis semiquantitatively, p94 was expressed in yeast as a hybrid protein between the DNA binding and activation domains of the yeast transcriptional activator Gal4. Transcriptional activation by the Gal4-p94:WT hybrid protein is precluded by p94 autolysis. Complete or partial loss of autolytic activity by C129S active site mutation, limb girdle muscular dystrophy type 2A ...Continue Reading

References

May 1, 1992·Proceedings of the National Academy of Sciences of the United States of America·B DasmahapatraJ Schwartz
Jul 15, 1991·Proceedings of the National Academy of Sciences of the United States of America·H D LiebigE Kuechler
May 1, 2001·Journal of Biochemistry·H Sorimachi, K Suzuki
Mar 21, 2002·The Journal of Biological Chemistry·Chiho FukiageThomas R Shearer
Jun 27, 2002·Proceedings of the National Academy of Sciences of the United States of America·M J SpencerJ S Beckmann
Oct 9, 2002·Trends in Biochemical Sciences·Peter Tompa
Jul 5, 2003·Physiological Reviews·Darrell E GollJinyang Cong
Nov 5, 2003·The Journal of Biological Chemistry·Yasuko OnoHiroyuki Sorimachi
Dec 4, 2003·Molecular and Cellular Biology·Mathieu TaveauIsabelle Richard
Jan 30, 2004·Diabetes·Koichi SuzukiHiroyuki Sorimachi
Apr 10, 2004·The Journal of Biological Chemistry·Beatriz Garcia DiazPeter L Davies
May 13, 2004·Human Molecular Genetics·I KramerovaMelissa J Spencer
Aug 2, 2005·Experimental Eye Research·M ShihT R Shearer
Aug 24, 2005·Human Molecular Genetics·Kimberly A HuebschGregory A Cox

❮ Previous
Next ❯

Citations

Jul 17, 2010·The Journal of Biological Chemistry·Jaakko SarparantaBjarne Udd
May 13, 2010·The Journal of Biological Chemistry·Yasuko OnoHiroyuki Sorimachi
Mar 4, 2009·The Journal of Biological Chemistry·Eugene FutaiShoichi Ishiura
May 12, 2012·Brain : a Journal of Neurology·Gerald PfefferPatrick F Chinnery
Sep 22, 2010·Human Molecular Genetics·Karine ChartonIsabelle Richard
Oct 2, 2009·Clinical and Experimental Pharmacology & Physiology·Robyn M Murphy
Oct 1, 2009·Physiological Reviews·Aikaterini Kontrogianni-KonstantopoulosRobert J Bloch
May 19, 2010·Circulation·Martin M LeWinter, Henk Granzier
Nov 19, 2008·PloS One·Amets SáenzAdolfo López de Munain
Jun 15, 2011·Proceedings of the Japan Academy. Series B, Physical and Biological Sciences·Hiroyuki SorimachiYasuko Ono
Sep 13, 2015·Biochimie·Yasuko OnoHiroyuki Sorimachi
Dec 17, 2014·Proceedings of the National Academy of Sciences of the United States of America·Yasuko OnoHiroyuki Sorimachi
Oct 30, 2010·Experimental Animals·Hiroyuki SorimachiYasuko Ono
Feb 8, 2011·Journal of Molecular Biology·Koichi OjimaHiroyuki Sorimachi
Nov 1, 2008·Neuromuscular Disorders : NMD·Jacques S Beckmann, Melissa Spencer
Sep 25, 2014·Genes to Cells : Devoted to Molecular & Cellular Mechanisms·Koichi OjimaHiroyuki Sorimachi
Apr 17, 2015·Human Molecular Genetics·Karine ChartonBjarne Udd
Oct 8, 2017·Journal of Muscle Research and Cell Motility·Patricia S PardoAladin M Boriek
Sep 27, 2018·The Journal of Biological Chemistry·Christian-Scott E McCartneyPeter L Davies
Sep 22, 2019·International Journal of Molecular Sciences·Jaione Lasa-ElgarrestaAinara Vallejo-Illarramendi
Apr 29, 2020·Neuropathology and Applied Neurobiology·M CerinoS Attarian
Mar 21, 2007·The Journal of Biological Chemistry·Koichi OjimaHiroyuki Sorimachi
Apr 8, 2020·Frontiers in Physiology·Thomas LanzicherHenk Granzier
Dec 22, 2011·Critical Reviews in Clinical Laboratory Sciences·Snezana KojicGeorgine Faulkner
Jun 5, 2020·International Journal of Molecular Sciences·Kiisa NishikawaDhruv Mishra
Nov 13, 2015·The Journal of Biological Chemistry·Eugene FutaiTaisuke Tomita
Feb 6, 2021·Biochimica Et Biophysica Acta. Proteins and Proteomics·Simone SpinozziIsabelle Richard
Jul 25, 2021·International Journal of Molecular Sciences·Anabel RicoAmets Sáenz
Aug 29, 2021·Journal of Genetics and Genomics = Yi Chuan Xue Bao·Shuyi ZhaoJinrong Peng

❮ Previous
Next ❯

Related Concepts

Related Feeds

ASBMB Publications

The American Society for Biochemistry and Molecular Biology (ASBMB) includes the Journal of Biological Chemistry, Molecular & Cellular Proteomics, and the Journal of Lipid Research. Discover the latest research from ASBMB here.