Protein degradation in mitochondria.

The biogenesis of mitochondria and the maintenance of mitochondrial functions depends on an autonomous proteolytic system in the organelle which is highly conserved throughout evolution. Components of this system include processing peptidases and ATP-dependent proteases, as well as molecular chaperone proteins and protein complexes with apparently regulatory functions. While processing peptidases mediate maturation of nuclear-encoded mitochondrial preproteins, quality control within various subcompartments of mitochondria is ensured by ATP-dependent proteases which selectively remove non-assembled or misfolded polypeptides. Moreover; these proteases appear to control the activity- or steady-state levels of specific regulatory proteins and thereby ensure mitochondrial genome integrity, gene expression and protein assembly.

[1]  T. Langer,et al.  AAA proteases: cellular machines for degrading membrane proteins. , 2000, Trends in biochemical sciences.

[2]  S. Gottesman,et al.  Posttranslational quality control: folding, refolding, and degrading proteins. , 1999, Science.

[3]  A. Ito,et al.  Mitochondrial processing peptidase: multiple-site recognition of precursor proteins. , 1999, Biochemical and biophysical research communications.

[4]  T. Langer,et al.  ATP-dependent proteases controlling mitochondrial function in the yeast Saccharomyces cerevisiae , 1999, Cellular and Molecular Life Sciences CMLS.

[5]  Nektarios Tavernarakis,et al.  The SPFH domain: implicated in regulating targeted protein turnover in stomatins and other membrane-associated proteins. , 1999, Trends in biochemical sciences.

[6]  J. Carrascosa,et al.  Mitochondrial localization and oligomeric structure of HClpP, the human homologue of E. coli ClpP. , 1999, Journal of molecular biology.

[7]  A. Horwich,et al.  Global unfolding of a substrate protein by the Hsp100 chaperone ClpA , 1999, Nature.

[8]  J. Adamec,et al.  Yeast and Human Frataxin Are Processed to Mature Form in Two Sequential Steps by the Mitochondrial Processing Peptidase* , 1999, The Journal of Biological Chemistry.

[9]  P. Kloetzel,et al.  The base of the proteasome regulatory particle exhibits chaperone-like activity , 1999, Nature Cell Biology.

[10]  A. Ballabio,et al.  Identification and characterization of AFG3L2, a novel paraplegin-related gene. , 1999, Genomics.

[11]  M. Yaffe,et al.  A Role for Ubiquitination in Mitochondrial Inheritance in Saccharomyces cerevisiae , 1999, The Journal of cell biology.

[12]  H. Stahlberg,et al.  Mitochondrial Lon of Saccharomyces cerevisiae is a ring-shaped protease with seven flexible subunits. , 1999, Proceedings of the National Academy of Sciences of the United States of America.

[13]  M. Hochstrasser,et al.  Substrate Targeting in the Ubiquitin System , 1999, Cell.

[14]  Walter Neupert,et al.  Prohibitins Regulate Membrane Protein Degradation by the m-AAA Protease in Mitochondria , 1999, Molecular and Cellular Biology.

[15]  W. Neupert,et al.  Chaperone-like activity of the AAA domain of the yeast Yme1 AAA protease , 1999, Nature.

[16]  E V Koonin,et al.  AAA+: A class of chaperone-like ATPases associated with the assembly, operation, and disassembly of protein complexes. , 1999, Genome research.

[17]  T. Hanekamp,et al.  YNT20, a bypass suppressor of yme1 yme2, encodes a putative 3′-5′ exonuclease localized in mitochondria of Saccharomyces cerevisiae , 1999, Current Genetics.

[18]  W. Schumann.  FtsH--a single-chain charonin? , 1999, FEMS microbiology reviews.

[19]  W. Neupert,et al.  Mcx1p, a ClpX homologue in mitochondria of Saccharomyces cerevisiae , 1998, FEBS Letters.

[20]  M. Bolotin-Fukuhara,et al.  A mutation in a novel yeast proteasomal gene, RPN11/MPR1, produces a cell cycle arrest, overreplication of nuclear and mitochondrial DNA, and an altered mitochondrial morphology. , 1998, Molecular biology of the cell.

[21]  A. Ito,et al.  Role of α-Subunit of Mitochondrial Processing Peptidase in Substrate Recognition* , 1998, The Journal of Biological Chemistry.

[22]  J. V. van Dijl,et al.  The ATPase and protease domains of yeast mitochondrial Lon: roles in proteolysis and respiration-dependent growth. , 1998, Proceedings of the National Academy of Sciences of the United States of America.

[23]  W. Neupert,et al.  The formation of respiratory chain complexes in mitochondria is under the proteolytic control of the m‐AAA protease , 1998, The EMBO journal.

[24]  P. Thorsness,et al.  Escape of mitochondrial DNA to the nucleus in yme1 yeast is mediated by vacuolar-dependent turnover of abnormal mitochondrial compartments. , 1998, Journal of cell science.

[25]  M. Yaffe,et al.  Prohibitin Family Members Interact Genetically with Mitochondrial Inheritance Components in Saccharomyces cerevisiae , 1998, Molecular and Cellular Biology.

[26]  Sergio Cocozza,et al.  Spastic Paraplegia and OXPHOS Impairment Caused by Mutations in Paraplegin, a Nuclear-Encoded Mitochondrial Metalloprotease , 1998, Cell.

[27]  A. Kihara,et al.  Different pathways for protein degradation by the FtsH/HflKC membrane-embedded protease complex: an implication from the interference by a mutant form of a new substrate protein, YccA. , 1998, Journal of molecular biology.

[28]  W. Neupert,et al.  The ATP-dependent PIM1 protease is required for the expression of intron-containing genes in mitochondria. , 1998, Genes & development.

[29]  P. Kloetzel,et al.  Evidence for a novel ATP-dependent protease from the rat liver mitochondrial intermembrane space: purification and characterisation. , 1998, Journal of biochemistry.

[30]  D. Markovitz,et al.  The human LON protease binds to mitochondrial promoters in a single-stranded, site-specific, strand-specific manner. , 1998, Biochemistry.

[31]  M. Latterich,et al.  The AAA team: related ATPases with diverse functions. , 1998, Trends in cell biology.

[32]  A. Ito,et al.  Role of alpha-subunit of mitochondrial processing peptidase in substrate recognition. , 1998, The Journal of biological chemistry.

[33]  W. Neupert,et al.  Autocatalytic processing of the ATP‐dependent PIM1 protease: crucial function of a pro‐region for sorting to mitochondria , 1997, The EMBO journal.

[34]  S. Gottesman,et al.  Regulatory Subunits of Energy-Dependent Proteases , 1997, Cell.

[35]  W Baumeister,et al.  Self-compartmentalizing proteases. , 1997, Trends in biochemical sciences.

[36]  A. Beyer.  Sequence analysis of the AAA protein family , 1997, Protein science : a publication of the Protein Society.

[37]  V. Géli,et al.  Functional cooperation of the mitochondrial processing peptidase subunits. , 1997, Journal of molecular biology.

[38]  H. Braun,et al.  The mitochondrial processing peptidase. , 1997, The international journal of biochemistry & cell biology.

[39]  D. J. Jamieson,et al.  The prohibitin family of mitochondrial proteins regulate replicative lifespan , 1997, Current Biology.

[40]  S. Bron,et al.  The chemistry and enzymology of the type I signal peptidases , 1997, Protein science : a publication of the Protein Society.

[41]  A. Kihara,et al.  Host regulation of lysogenic decision in bacteriophage lambda: transmembrane modulation of FtsH (HflB), the cII degrading protease, by HflKC (HflA). , 1997, Proceedings of the National Academy of Sciences of the United States of America.

[42]  L. Grivell,et al.  ATP-dependent proteases that also chaperone protein biogenesis. , 1997, Trends in biochemical sciences.

[43]  D. Markovitz,et al.  Bacterial Protease Lon Is a Site-specific DNA-binding Protein* , 1997, The Journal of Biological Chemistry.

[44]  Koreaki Ito,et al.  A protease complex in the Escherichia coli plasma membrane: HflKC (HflA) forms a complex with FtsH (HflB), regulating its proteolytic activity against SecY. , 1996, The EMBO journal.

[45]  L. Grivell,et al.  The role of protein degradation in mitochondrial function and biogenesis , 1996, Current Genetics.

[46]  W. Neupert,et al.  The molecular chaperone Hsp78 confers compartment-specific thermotolerance to mitochondria , 1996, The Journal of cell biology.

[47]  G. Mannhaupt,et al.  AAA proteases with catalytic sites on opposite membrane surfaces comprise a proteolytic system for the ATP‐dependent degradation of inner membrane proteins in mitochondria. , 1996, The EMBO journal.

[48]  S. Lindquist,et al.  HSP100/Clp proteins: a common mechanism explains diverse functions. , 1996, Trends in biochemical sciences.

[49]  Walter Neupert,et al.  The YTA10–12 Complex, an AAA Protease with Chaperone-like Activity in the Inner Membrane of Mitochondria , 1996, Cell.

[50]  R. Glockshuber,et al.  Substitution of PIM1 Protease in Mitochondria by Escherichia coli Lon Protease (*) , 1996, The Journal of Biological Chemistry.

[51]  L. Grivell,et al.  Afg3p, a mitochondrial ATP‐dependent metalloprotease, is involved in degradation of mitochondrially‐encoded Cox1, Cox3, Cob, Su6, Su8 and Su9 subunits of the inner membrane complexes III, IV and V , 1996, FEBS letters.

[52]  T. Hanekamp,et al.  Biochemical and functional analysis of the YME1 gene product, an ATP and zinc-dependent mitochondrial protease from S. cerevisiae. , 1996, Molecular biology of the cell.

[53]  P. Bross,et al.  Human ClpP protease: cDNA sequence, tissue‐specific expression and chromosomal assignment of the gene , 1995, FEBS letters.

[54]  S. Branda,et al.  Prediction and Identification of New Natural Substrates of the Yeast Mitochondrial Intermediate Peptidase (*) , 1995, The Journal of Biological Chemistry.

[55]  A. Tzagoloff,et al.  Mutations in RCA1 and AFG3 inhibit F1‐ATPase assembly in Saccharomyces cerevisiae , 1995, FEBS letters.

[56]  F. Sherman,et al.  Degradation of Cytochrome Oxidase Subunits in Mutants of Yeast Lacking Cytochrome c and Suppression of the Degradation by Mutation of yme1(*) , 1995, The Journal of Biological Chemistry.

[57]  Y. Fujiki,et al.  Multiple genes, including a member of the AAA family, are essential for degradation of unassembled subunit 2 of cytochrome c oxidase in yeast mitochondria , 1995, Molecular and cellular biology.

[58]  J. W. Chase,et al.  Mutations in the mitochondrial ATP synthase gamma subunit suppress a slow-growth phenotype of yme1 yeast lacking mitochondrial DNA. , 1995, Genetics.

[59]  A. Horwich.  Molecular Chaperones: Resurrection or destruction? , 1995, Current Biology.

[60]  E. Jupe,et al.  Prohibitin: Potential role in senescence, development, and tumor suppression , 1995, Experimental Gerontology.

[61]  A. Barrett,et al.  Characterization of a Mitochondrial Metallopeptidase Reveals Neurolysin as a Homologue of Thimet Oligopeptidase (*) , 1995, The Journal of Biological Chemistry.

[62]  R. Taussig,et al.  Mammalian Membrane-bound Adenylyl Cyclases (*) , 1995, The Journal of Biological Chemistry.

[63]  A. Horwich.  Resurrection or destruction? Recent studies implicate Hspl 04/Clp family chaperones in both protein disaggregation and protein degradation. How do these homologous ring-shaped complexes function in such different ways? , 1995 .

[64]  L. Grivell.  Nucleo-mitochondrial interactions in mitochondrial gene expression. , 1995, Critical reviews in biochemistry and molecular biology.

[65]  F. Kalousek,et al.  Mitochondrial intermediate peptidase. , 1995, Methods in enzymology.

[66]  W. Neupert,et al.  Purification and characterization of mitochondrial processing peptidase of Neurospora crassa. , 1995, Methods in enzymology.

[67]  S. Gottesman,et al.  A molecular chaperone, ClpA, functions like DnaK and DnaJ. , 1994, Proceedings of the National Academy of Sciences of the United States of America.

[68]  M. Gottesman,et al.  Synthesis, processing, and localization of human Lon protease. , 1994, The Journal of biological chemistry.

[69]  W. Neupert,et al.  Molecular chaperones cooperate with PIM1 protease in the degradation of misfolded proteins in mitochondria. , 1994, The EMBO journal.

[70]  J. Jang,et al.  A new member of a family of ATPases is essential for assembly of mitochondrial respiratory chain and ATP synthetase complexes in Saccharomyces cerevisiae. , 1994, The Journal of biological chemistry.

[71]  H. Feldmann,et al.  Yta10p, a member of a novel ATPase family in yeast, is essential for mitochondrial function , 1994, FEBS letters.

[72]  L. Grivell,et al.  Yeast sequencing reports. Sequence of the AFG3 gene encoding a new member of the FtsH/Yme1/Tma subfamily of the AAA‐protein family , 1994, Yeast.

[73]  H. Feldmann,et al.  Identification of a set of yeast genes coding for a novel family of putative ATPases with high similarity to constituents of the 26S protease complex , 1994, Yeast.

[74]  K. H. White,et al.  Mitochondrial morphological and functional defects in yeast caused by yme1 are suppressed by mutation of a 26S protease subunit homologue. , 1994, Molecular biology of the cell.

[75]  R. A. Rollins,et al.  MIP1, a new yeast gene homologous to the rat mitochondrial intermediate peptidase gene, is required for oxidative metabolism in Saccharomyces cerevisiae. , 1994, Molecular and cellular biology.

[76]  G. Schatz,et al.  Requirement for the yeast gene LON in intramitochondrial proteolysis and maintenance of respiration. , 1994, Science.

[77]  A. Schneider,et al.  Purified inner membrane protease I of yeast mitochondria is a heterodimer. , 1994, The Journal of biological chemistry.

[78]  F. Sherman,et al.  PIM1 encodes a mitochondrial ATP-dependent protease that is required for mitochondrial function in the yeast Saccharomyces cerevisiae. , 1994, The Journal of biological chemistry.

[79]  D. Wolf,et al.  Proteinase yscD (oligopeptidase yscD). Structure, function and relationship of the yeast enzyme with mammalian thimet oligopeptidase (metalloendopeptidase, EP 24.15). , 1994, European journal of biochemistry.

[80]  T. Fox,et al.  A mitochondrial protease with two catalytic subunits of nonoverlapping specificities. , 1993, Science.

[81]  M. Willingham,et al.  A human mitochondrial ATP-dependent protease that is highly homologous to bacterial Lon protease. , 1993, Proceedings of the National Academy of Sciences of the United States of America.

[82]  T L Mason,et al.  HSP78 encodes a yeast mitochondrial heat shock protein in the Clp family of ATP-dependent proteases , 1993, Molecular and cellular biology.

[83]  K. H. White,et al.  Inactivation of YME1, a Member of the ftsH-SEC18-PAS1-CDC48 Family of Putative ATPase-Encoding Genes, Causes Increased Escape of DNA from Mitochondria in Saccharomyces cerevisiae , 1993, Molecular and cellular biology.

[84]  S. Tsuboi,et al.  Autophagy in yeast demonstrated with proteinase-deficient mutants and conditions for its induction , 1992, The Journal of cell biology.

[85]  L. Rosenberg,et al.  Rat liver mitochondrial intermediate peptidase (MIP): purification and initial characterization. , 1992, The EMBO journal.

[86]  J. R. Smith,et al.  Prohibitin, an evolutionarily conserved intracellular protein that blocks DNA synthesis in normal fibroblasts and HeLa cells , 1991, Molecular and cellular biology.

[87]  A. Schneider,et al.  Inner membrane protease I, an enzyme mediating intramitochondrial protein sorting in yeast. , 1991, The EMBO journal.

[88]  P. Thorsness,et al.  Escape of DNA from mitochondria to the nucleus in Saccharomyces cerevisiae , 1990, Nature.

[89]  M. Costanzo,et al.  Control of mitochondrial gene expression in Saccharomyces cerevisiae. , 1990, Annual review of genetics.

[90]  J. R. Smith,et al.  Isolation of a cDNA that hybrid selects antiproliferative mRNA from rat liver. , 1989, Biochemical and biophysical research communications.