Type I collagen in Hsp47-null cells is aggregated in endoplasmic reticulum and deficient in N-propeptide processing and fibrillogenesis

Molecular Biology of the Cell
Yoshihito IshidaKazuhiro Nagata

Abstract

Heat-shock protein of 47 kDa (Hsp47) is a molecular chaperone that recognizes collagen triple helices in the endoplasmic reticulum (ER). Hsp47-knockout mouse embryos are deficient in the maturation of collagen types I and IV, and collagen triple helices formed in the absence of Hsp47 show increased susceptibility to protease digestion. We show here that the fibrils of type I collagen produced by Hsp47-/- cells are abnormally thin and frequently branched. Type I collagen was highly accumulated in the ER of Hsp47-/- cells, and its secretion rate was much slower than that of Hsp47+/+ cells, leading to accumulation of the insoluble aggregate of type I collagen within the cells. Transient expression of Hsp47 in the Hsp47-/- cells restored normal extracellular fibril formation and intracellular localization of type I collagen. Intriguingly, type I collagen with unprocessed N-terminal propeptide (N-propeptide) was secreted from Hsp47-/- cells and accumulated in the extracellular matrix. These results indicate that Hsp47 is required for correct folding and prevention of aggregation of type I collagen in the ER and that this function is indispensable for efficient secretion, processing, and fibril formation of collagen.

References

Jan 1, 1978·Annual Review of Biochemistry·J H Fessler, L I Fessler
Jan 1, 1991·Annual Review of Biophysics and Biophysical Chemistry·J Engel, D J Prockop
Apr 1, 1991·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·H KuivaniemiD J Prockop
Jul 1, 1986·The Journal of Cell Biology·K NagataK M Yamada
Jan 1, 1985·Journal of Cellular Biochemistry·L PeltonenL Ryhänen
Jan 1, 1974·Journal of Supramolecular Structure·L I FesslerJ H Fessler
Jul 1, 1982·European Journal of Biochemistry·L Tuderman, D J Prockop
Jan 1, 1995·Annual Review of Biochemistry·D J Prockop, K I Kivirikko
May 14, 1996·Proceedings of the National Academy of Sciences of the United States of America·S W LiD J Prockop
May 23, 1998·The Journal of Biological Chemistry·R WilsonN J Bulleid
May 18, 1999·The Journal of Biological Chemistry·A R WalmsleyN J Bulleid
Jul 21, 2000·The Biochemical Journal·C A Thomson, V S Ananthanarayanan
Dec 26, 2001·The Journal of Biological Chemistry·Takaki KoideKazuhiro Nagata
Feb 21, 2002·The Journal of Cell Biology·Franck PerezBruno Goud
Jun 18, 2002·Journal of Structural Biology·David J S Hulmes
Jul 13, 2002·The Journal of Biological Chemistry·Mohammed TasabNeil J Bulleid
Apr 26, 2003·Matrix Biology : Journal of the International Society for Matrix Biology·Johanna Myllyharju
Dec 31, 2003·Trends in Genetics : TIG·Johanna Myllyharju, Kari I Kivirikko
Feb 28, 2004·Seminars in Cell & Developmental Biology·Kazuhiro Nagata

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Citations

Jun 10, 2011·Cellular and Molecular Life Sciences : CMLS·Ewa Szolajska, Jadwiga Chroboczek
Feb 26, 2013·Cell Stress & Chaperones·Tomoyuki KakugawaShigeru Kohno
Sep 22, 2009·Nature Cell Biology·Tomohiko MurakamiKazunori Imaizumi
Jun 15, 2011·Nature Reviews. Endocrinology·Antonella ForlinoJoan C Marini
Aug 1, 2012·Proceedings of the National Academy of Sciences of the United States of America·Christine WidmerUlrich Baumann
Jan 12, 2012·The Journal of Biological Chemistry·Takashi OnoKazuhiro Nagata
May 15, 2013·Annual Review of Genomics and Human Genetics·Maria Antonietta De MatteisCathal Wilson
Jan 24, 2012·Reproductive Sciences·Jerome F Strauss
Nov 22, 2013·Respiratory Research·Tomoyuki KakugawaShigeru Kohno
Sep 19, 2007·Genome Biology·Paul M Krzyzanowski, Miguel A Andrade-Navarro
Jan 13, 2009·Journal of Lipid Research·Mark H DoolittleHoward Wong
Jul 25, 2009·PLoS Genetics·Cord DrögemüllerTosso Leeb
Dec 10, 2009·PLoS Genetics·Jae Won ChoiRichard J Bram
Nov 5, 2010·PloS One·Daisuke HasegawaToshihiro Nakajima
Apr 30, 2014·Human Molecular Genetics·Caressa D LietmanDeborah Krakow
Nov 14, 2012·Annual Review of Genetics·Peter H Byers, Shawna M Pyott
Jan 12, 2010·Journal of Biomedical Optics·Doris HelbigUwe Paasch
Dec 26, 2006·Expert Review of Anticancer Therapy·Joseph G Sinkovics
May 25, 2011·The Journal of Cell Biology·Deanna G WilsonMark J Solloway
Jan 29, 2016·Biochemical and Biophysical Research Communications·Isao KiiAkira Kudo
Dec 30, 2015·Journal of Tissue Engineering and Regenerative Medicine·Martin VielreicherOliver Friedrich
Dec 25, 2010·Trends in Cell Biology·Elena MakareevaSergey Leikin
Dec 15, 2010·Biochemical and Biophysical Research Communications·Hiroshi KitamuraTsutomu Chiba
Sep 25, 2010·International Journal of Cardiology·Mitsuyo MachidaNanako Kawaguchi
Dec 16, 2006·Trends in Molecular Medicine·Takashi Taguchi, M Shawkat Razzaque
Jun 6, 2007·Traffic·Vytaute Starkuviene, Rainer Pepperkok
Oct 24, 2006·Genes to Cells : Devoted to Molecular & Cellular Mechanisms·Asami OguroYoriko Atomi
Sep 15, 2010·Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research·Brian P KelleyBrendan Lee
Jan 14, 2011·Skin Research and Technology : Official Journal of International Society for Bioengineering and the Skin (ISBS) [and] International Society for Digital Imaging of Skin (ISDIS) [and] International Society for Skin Imaging (ISSI)·Doris Helbig, Uwe Paasch
Jun 25, 2010·International Journal of Cosmetic Science·D HelbigU Paasch
Oct 15, 2013·Clinical Oral Implants Research·Ghadeer N ThaljiLyndon F Cooper
Sep 18, 2007·Cardiovascular Pathology : the Official Journal of the Society for Cardiovascular Pathology·W David MerrymanMichael S Sacks

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