Translating m-AAA protease function in mitochondria to hereditary spastic paraplegia

Trends in Molecular Medicine
Elena I Rugarli, Thomas Langer

Abstract

Hereditary spastic paraplegia (HSP) is a genetically heterogeneous neurodegenerative disorder that is characterized by progressive and cell-specific axonal degeneration. An autosomal recessive form of the disease is caused by mutations in paraplegin, which is a conserved subunit of the ubiquitous and ATP-dependent m-AAA protease in mitochondria. The m-AAA protease carries out protein quality control in the inner membrane of the mitochondria, suggesting a pathogenic role of misfolded proteins in HSP. A recent study demonstrates that the m-AAA protease regulates ribosome assembly and translation within mitochondria by controlling proteolytic maturation of a ribosomal subunit. Here, we will discuss implications of the dual role of the m-AAA protease in protein activation and degradation for mitochondrial dysfunction and axonal degeneration.

References

Jul 1, 1996·Frontiers in Bioscience : a Journal and Virtual Library·P J Hollenbeck
Jun 23, 1998·American Journal of Human Genetics·G De MicheleS Cocozza
Apr 27, 2000·Trends in Biochemical Sciences·T Langer
Aug 31, 2000·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Y KanaiN Hirokawa
Jul 23, 2002·Nature Genetics·Heema PatelAndrew H Crosby
Oct 2, 2002·American Journal of Human Genetics·Andrew H Crosby, Christos Proukakis
Oct 2, 2002·American Journal of Human Genetics·Evan ReidDouglas A Marchuk
Mar 11, 2003·Nature Genetics·Imke PulsKenneth H Fischbeck
Sep 26, 2003·American Journal of Human Genetics·Shirley RainierJohn K Fink
Oct 18, 2003·American Journal of Human Genetics·Michael A SimpsonAndrew H Crosby
Nov 5, 2003·Experimental Neurology·John K Fink
Dec 23, 2003·The Journal of Biological Chemistry·Mingsheng Zhang, Philip Coffino
Jan 15, 2004·The Journal of Clinical Investigation·Fatima FerreirinhaElena I Rugarli
Feb 27, 2004·Brain : a Journal of Neurology·Philip A WilkinsonThomas T Warner
May 8, 2004·Trends in Cell Biology·Erika L F Holzbaur
May 20, 2004·Journal of Cell Science·Kyle E Miller, Michael P Sheetz
Jul 1, 2004·The Journal of Cell Biology·Julia M EdgarIan R Griffiths

❮ Previous
Next ❯

Citations

Dec 7, 2011·Acta Neuropathologica·Mariusz Karbowski, Albert Neutzner
May 24, 2011·Journal of Neurology·Massimiliano FilostoAlessandro Padovani
Dec 4, 2009·Proceedings of the National Academy of Sciences of the United States of America·Christoph BieniossekUlrich M Baumann
Aug 7, 2009·Molecular Biology of the Cell·Mirko KoppenThomas Langer
Oct 16, 2012·Brain : a Journal of Neurology·Stephan KlebeAlexandra Durr
Mar 18, 2009·Human Molecular Genetics·Paola MartinelliElena I Rugarli
Jun 2, 2011·Cold Spring Harbor Perspectives in Biology·Michael J BakerThomas Langer
Nov 13, 2008·Journal of Medical Genetics·P Yu-Wai-ManP F Chinnery
Oct 21, 2009·PloS One·Tobias KarlbergHerwig Schüler
Jan 18, 2007·Proceedings of the National Academy of Sciences of the United States of America·Mats I EkstrandNils-Göran Larsson
Apr 17, 2010·Journal of Biomedicine & Biotechnology·Paulien SmitsLambert van den Heuvel
Jun 24, 2014·Experimental Neurology·Temistocle Lo GiudiceAntonio Orlacchio
Jun 15, 2007·Critical Reviews in Biochemistry and Molecular Biology·Mirko Koppen, Thomas Langer
Aug 3, 2011·Annals of Medicine·Emil Ylikallio, Anu Suomalainen
Jan 16, 2007·Molecular and Cellular Endocrinology·Zvi GranotJoseph Orly
Sep 22, 2015·Molecular Cell·Santhanam ShanmughapriyaMuniswamy Madesh
Nov 22, 2011·Molecular Aspects of Medicine·Diego SbardellaMassimo Coletta
Nov 30, 2010·Neuromuscular Disorders : NMD·Z M A Chrzanowska-LightowlersR N Lightowlers
Dec 18, 2015·Biochimica Et Biophysica Acta·Yang Zhang, Michael R Maurizi
Feb 24, 2016·Biochimica Et Biophysica Acta·Lucia BiasuttoMario Zoratti
Jan 25, 2008·The EMBO Journal·Takashi Tatsuta, Thomas Langer
Sep 28, 2007·Traffic·Susan Roehl White, Brett Lauring
Feb 27, 2010·Human Mutation·Florian BonnAshraf U Mannan
Jan 29, 2010·Movement Disorders : Official Journal of the Movement Disorder Society·Tobias WarneckePeter Young
Jun 12, 2012·Journal of Structural Biology·L'uboš AmbroEva Kutejová
Jun 11, 2010·Essays in Biochemistry·Michael R Duchen, Gyorgy Szabadkai
Jul 4, 2012·Biochimica Et Biophysica Acta·Ruchika AnandMichael James Baker
Jun 10, 2015·Acta Crystallographica. Section D, Biological Crystallography·Marina VostrukhinaUlrich Baumann
Mar 10, 2009·Biochimica Et Biophysica Acta·Valerio CarelliAlfredo A Sadun
May 27, 2006·Biochimica Et Biophysica Acta·Mafalda Escobar-Henriques, Thomas Langer
Sep 22, 2007·Current Opinion in Neurology·Massimo Zeviani, Valerio Carelli
Jun 10, 2008·The Biochemical Journal·Philip A Robinson
Jan 1, 2015·Human Genome Variation·Hiroyuki YahikozawaNaomichi Matsumoto
Jan 31, 2021·Communications Biology·Philippe FernandesValeria Naim
Nov 30, 2006·The Journal of Biological Chemistry·Hiroaki SuzukiNono Takeuchi
Aug 2, 2021·Journal of the Neurological Sciences·Han-Lin ChiangYi-Chung Lee
Dec 14, 2021·Frontiers in Molecular Biosciences·Liena E O ElsayedGiovanni Stevanin

❮ Previous
Next ❯

Related Concepts

Related Feeds

Cell-Type Specific Viral Vectors

Viral vectors are used in biological research and therapy to deliver genetic material into cells. However, the efficiency of viral vectors varies depending on the cell type. Here is the latest research on cell-type-specific viral vectors.

Cell-Type-Specific Viral Vectors (ASM)

Viral vectors are used in biological research and therapy to deliver genetic material into cells. However, the efficiency of viral vectors varies depending on the cell type. Here is the latest research on cell-type-specific viral vectors.

Cell-Type-Specific Viral Vectors

Viral vectors are used in biological research and therapy to deliver genetic material into cells. However, the efficiency of viral vectors varies depending on the cell type. Here is the latest research on cell-type-specific viral vectors.