3.4.24.64: mitochondrial processing peptidase

This is an abbreviated version!
For detailed information about mitochondrial processing peptidase, go to the full flat file.

Word Map on EC 3.4.24.64

Reaction

Release of N-terminal targetting peptides from precursor proteins imported into the mitochondrion, typically with Arg in position P2 =

Synonyms

More, Processing enhancing peptidase, Proteinase, mitochondrial protein precursor-processing, Matrix peptidase, Matrix processing peptidase, Matrix processing proteinase, Mitochondrial protein precursor-processing proteinase, MPP, Mitochondrial chelator-sensitive protease, General mitochondrial processing peptidase, TPP, processing peptidase, Alpha-MPP, Beta-MPP, HA1523, P-52, P-55, PEP, processing enhancing protein, Mas1, Mas2, Plsp1, Atp23, inner membrane peptidase processing enzyme, Imp1, Imp2, mitochondrial processing peptidase, HPP, GPP, TTHA1264, thylakoidal processing peptidase, Plsp2, MPP-alpha, MMP-beta, mitochondrial processing peptidase-alpha protein, PMPCA, mitochondrial processing protease

ECTree

     3 Hydrolases
         3.4 Acting on peptide bonds (peptidases)
             3.4.24 Metalloendopeptidases
                3.4.24.64 mitochondrial processing peptidase

Reference

Reference on EC 3.4.24.64 - mitochondrial processing peptidase

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REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Rawlings, N.D.; Barrett, A.J.
Homologues of insulinase, a new superfamily of metalloendopeptidases
Biochem. J.
275
389-391
1991
Saccharomyces cerevisiae, Neurospora crassa
Manually annotated by BRENDA team
Kalousek, F.; Neupert, W.; Omura, T.; Schatz, G.; Schmitz, U.K.
Uniform nomenclature for the mitochondrial peptidases cleaving precursors of mitochondrial proteins
Trends Biochem. Sci.
18
249
1993
Saccharomyces cerevisiae, Neurospora crassa, Rattus norvegicus, Solanum tuberosum
Manually annotated by BRENDA team
Bhni, P.C.; Daum, G.
Processing of mitochondrial polypeptide precursors in yeast
Methods Enzymol.
97
311-323
1983
Saccharomyces cerevisiae, Saccharomyces cerevisiae D-273-10B
Manually annotated by BRENDA team
McAda, P.C.; Douglas, M.G.
A yeast mitochondrial chelator-sensitive protease that processes cytoplasmically synthesized protein precursors: isolation from yeast and assay
Methods Enzymol.
97
337-344
1983
Saccharomyces cerevisiae
Manually annotated by BRENDA team
Bhni, P.C.; Daum, G.; Schatz, G.
Import of proteins into mitochondria. Partial purification of a matrix-located protease involved in cleavage of mitochondrial precursor polypeptides
J. Biol. Chem.
258
4937-4943
1983
Saccharomyces cerevisiae, Saccharomyces cerevisiae D-273-10B
Manually annotated by BRENDA team
Miura, S.; Amaya, Y.; Mori, M.
A metalloprotease involved in the processing of mitochondrial precursor proteins
Biochem. Biophys. Res. Commun.
134
1151-1159
1986
Rattus norvegicus
Manually annotated by BRENDA team
Kumamoto, T.; Omura, T.
Characterization of a mitochondrial matrix protease catalyzing the processing of adrenodoxin precursor
J. Biochem.
100
247-254
1986
Bos taurus, Rattus norvegicus
Manually annotated by BRENDA team
Hawlitschek, G.; Schneider, H.; Schmidt, B.; Tropschug, M.; Hartl, F.U.; Neupert, W.
Mitochondrial protein import: identification of processing peptidase and of PEP, a processing enhancing protein
Cell
53
795-806
1988
Neurospora crassa
Manually annotated by BRENDA team
Yang, M.; Jensen, R.E.; Yaffe, M.P.; Oppliger, W.; Schatz, G.
Import of proteins into yeast mitochondria: the purified matrix processing protease contains two subunits which are encoded by the nuclear MAS1 and MAS2 genes
EMBO J.
7
3857-3862
1988
Saccharomyces cerevisiae, Saccharomyces cerevisiae D-273-10B
Manually annotated by BRENDA team
Jensen, R.E.; Yaffe, M.P.
Import of proteins into yeast mitochondria: the nuclear MAS2 gene encodes a component of the processing protease that is homologous to the MAS1-encoded subunit
EMBO J.
7
3863-3871
1988
Saccharomyces cerevisiae
Manually annotated by BRENDA team
Witte, C.; Jensen, R.E.; Yaffe, M.P.; Schatz, G.
MAS1, a gene essential for yeast mitochondrial assembly, encodes a subunit of the mitochondrial processing protease
EMBO J.
7
1439-1447
1988
Saccharomyces cerevisiae, More
Manually annotated by BRENDA team
Kleiber, J.; Kalousek, F.; Swaroop, M.; Rosenberg, L.E.
The general mitochondrial matrix processing protease from rat liver: structural characterization of the catalytic subunit
Proc. Natl. Acad. Sci. USA
87
7978-7982
1990
More, Rattus norvegicus
Manually annotated by BRENDA team
Geli, V.; Yang, M.; Suda, K.; Lustig, A.; Schatz, G.
The MAS-encoded processing protease of yeast mitochondria. Overproduction and characterization of its two nonidentical subunits
J. Biol. Chem.
265
19216-19222
1990
Saccharomyces cerevisiae
Manually annotated by BRENDA team
Schneider, H.; Arretz, M.; Wachter, E.; Neupert, W.
Matrix processing peptidase of mitochondria. Structure-function relationships
J. Biol. Chem.
265
9881-9887
1990
More, Neurospora crassa
Manually annotated by BRENDA team
Isaya, G.; Kalousek, F.; Fenton, W.A.; Rosenberg, L.E.
Cleavage of precursors by the mitochondrial processing peptidase requires a compatible mature protein or an intermediate octapeptide
J. Cell Biol.
113
65-76
1991
Rattus norvegicus
Manually annotated by BRENDA team
Yang, M.; Geli, V.; Oppliger, W.; Suda, K.; James, P.; Schatz, G.
The MAS-encoded processing protease of yeast mitochondria. Interaction of the purified enzyme with signal peptides and a purified precursor protein
J. Biol. Chem.
266
6416-6423
1991
Saccharomyces cerevisiae
Manually annotated by BRENDA team
Eriksson, A.C.; Glaser, E.
Mitochondrial processing proteinase: a general processing proteinase of spinach leaf mitochondria is a membrane-bound enzyme
Biochim. Biophys. Acta
1140
208-214
1992
More, Solanum tuberosum, Spinacia oleracea
-
Manually annotated by BRENDA team
Emmermann, M.; Schmitz, U.K.
The cytochrome c reductase integrated processing peptidase from potato mitochondria belongs to a new class of metalloendoproteases
Plant Physiol.
103
615-620
1993
Solanum tuberosum
Manually annotated by BRENDA team
Emmermann, M.; Braun, H.P.; Arretz, M.; Schmitz, U.K.
Characterization of the bifunctional cytochrome c reductase-processing peptidase complex from potato mitochondria
J. Biol. Chem.
268
18936-18942
1993
More, Solanum tuberosum
Manually annotated by BRENDA team
Saavedra-Alanis, V.M.; Rysavy, P.; Rosenberg, L.E.; Kalousek, F.
Rat liver mitochondrial processing peptidase. Both alpha- and beta-subunits are required for activity
J. Biol. Chem.
269
9284-9288
1994
Rattus norvegicus
Manually annotated by BRENDA team
Arretz, M.; Schneider, H.; Guiard, B.; Brunner, M.; Neupert, W.
Characterization of the mitochondrial processing peptidase of Neurospora crassa
J. Biol. Chem.
269
4959-4967
1994
Neurospora crassa
Manually annotated by BRENDA team
Geli, V.
Functional reconstitution in Escherichia coli of the yeast mitochondrial matrix peptidase from its two inactive subunits
Proc. Natl. Acad. Sci. USA
90
6247-6251
1993
Saccharomyces cerevisiae
Manually annotated by BRENDA team
Sjling, S.; Eriksson, A.C.; Glaaser, E.
A helical element in the C-terminal domain of the N. plumbaginifolia F1 beta presequence is important for recognition by the mitochondrial processing peptidase
J. Biol. Chem.
269
32059-32062
1994
Spinacia oleracea
Manually annotated by BRENDA team
Bassham, D.C.; Creighton, A.M.; Arretz, M.; Brunner, M.; Robinson, C.
Efficient but aberrant cleavage of mitochondrial precursor proteins by the chloroplast stromal processing peptidase
Eur. J. Biochem.
221
523-528
1994
Neurospora crassa
Manually annotated by BRENDA team
Brunner, M.; Neupert, W.
Purification and characterization of mitochondrial processing peptidase of Neurospora crassa
Methods Enzymol.
248
717-728
1995
Neurospora crassa
Manually annotated by BRENDA team
Braun, H.P.; Schmitz, U.K.
The bifunctional cytochrome c reductase/processing peptidase complex from plant mitochondria
J. Bioenerg. Biomembr.
27
423-436
1995
Solanum tuberosum, Spinacia oleracea, Triticum aestivum
Manually annotated by BRENDA team
Waltner, M.; Weiner, H.
Conversion of a nonprocessed mitochondrial precursor protein into one that is processed by the mitochondrial processing peptidase
J. Biol. Chem.
270
26311-26317
1995
Rattus norvegicus
Manually annotated by BRENDA team
Gakh, O.; Obsil, T.; Adamec, J.; Spizek, J.; Amler, E.; Janata, J.; Kalousek, F.
Substrate binding changes conformation of the alpha-, but not the beta-subunit of mitochondrial processing peptidase
Arch. Biochem. Biophys.
385
392-396
2001
Saccharomyces cerevisiae
Manually annotated by BRENDA team
Kitada, S.; Kojima, K.; Ito, A.
Glu191 and Asp195 in rat mitochondrial processing peptidase beta subunit are involved in effective cleavage of precursor protein through interaction with the proximal arginine
Biochem. Biophys. Res. Commun.
287
594-599
2001
Rattus norvegicus
Manually annotated by BRENDA team
Braun, H.P.; Schmitz, U.K.
The mitochondrial processing peptidase
Int. J. Biochem. Cell Biol.
29
1043-1045
1997
Saccharomyces cerevisiae, Neurospora sp., Rattus norvegicus, Solanum tuberosum, Triticum aestivum
Manually annotated by BRENDA team
Song, M.C.; Ogishima, T.; Ito, A.
Importance of residues carboxyl terminal relative to the cleavage site in substrates of mitochondrial processing peptidase for their specific recognition and cleavage
J. Biochem.
124
1045-1049
1998
Bos taurus
Manually annotated by BRENDA team
Deng, K.; Zhang, L.; Kachurin, A.M.; Yu, L.; Xia, D.; Kim, H.; Deisenhofer, J.; Yu, C.A.
Activation of a matrix processing peptidase from the crystalline cytochrome bc1 complex of bovine heart mitochondria
J. Biol. Chem.
273
20752-20757
1998
Bos taurus
Manually annotated by BRENDA team
Shimokata, K.; Kitada, S.; Ogishima, T.; Ito, A.
Role of alpha-subunit of mitochondrial processing peptidase in substrate recognition
J. Biol. Chem.
273
25158-25163
1998
Saccharomyces cerevisiae
Manually annotated by BRENDA team
Kojima, K.; Kitada, S.; Shimokata, K.; Ogishima, T.; Ito, A.
Cooperative formation of a substrate binding pocket by alpha- and beta-subunits of mitochondrial processing peptidase
J. Biol. Chem.
273
32542-32546
1998
Saccharomyces cerevisiae
Manually annotated by BRENDA team
Kitada, S.; Kojima, K.; Shimokata, K.; Ogishima, T.; Ito, A.
Glutamate residues required for substrate binding and cleavage activity in mitochondrial processing peptidase
J. Biol. Chem.
273
32547-32553
1998
Saccharomyces cerevisiae, Rattus norvegicus
Manually annotated by BRENDA team
Kojima, K.; Kitada, S.; Ogishima, T.; Ito, A.
A proposed common structure of substrates bound to mitochondrial processing peptidase
J. Biol. Chem.
276
2115-2121
2001
Saccharomyces cerevisiae
Manually annotated by BRENDA team
Kitada, S.; Yamasaki, E.; Kojima, K.; Ito, A.
Determination of the cleavage site of the presequence by mitochondrial processing peptidase on the substrate binding scaffold and the multiple subsites inside a molecular cavity
J. Biol. Chem.
278
1879-1885
2003
Saccharomyces cerevisiae, Rattus norvegicus
Manually annotated by BRENDA team
Taylor, A.B.; Smith, B.S.; Kitada, S.; Kojima, K.; Miyaura, H.; Otwinowski, Z.; Ito, A.; Deisenhofer, J.
Crystal structures of mitochondrial processing peptidase reveal the mode for specific cleavage of import signal sequences
Structure
9
615-625
2001
Saccharomyces cerevisiae
Manually annotated by BRENDA team
Janata, J.; Hola, K.; Kubala, M.; Gakh, O.; Parkhomenko, N.; Matuskova, A.; Kutejova, E.; Amler, E.
Substrate evokes translocation of both domains in the mitochondrial processing peptidase alpha-subunit during which the C-terminus acts as a stabilizing element
Biochem. Biophys. Res. Commun.
316
211-217
2004
Saccharomyces cerevisiae
Manually annotated by BRENDA team
Oshima, T.; Yamasaki, E.; Ogishima, T.; Kadowaki, K.; Ito, A.; Kitada, S.
Recognition and processing of a nuclear-encoded polyprotein precursor by mitochondrial processing peptidase
Biochem. J.
385
755-761
2005
Saccharomyces cerevisiae
Manually annotated by BRENDA team
Nomura, H.; Athauda, S.B.; Wada, H.; Maruyama, Y.; Takahashi, K.; Inoue, H.
Identification and reverse genetic analysis of mitochondrial processing peptidase and the core protein of the cytochrome bc1 complex of Caenorhabditis elegans, a model parasitic nematode
J. Biochem.
139
967-979
2006
Brugia malayi, Caenorhabditis elegans
Manually annotated by BRENDA team
Rudhe, C.; Clifton, R.; Chew, O.; Zemam, K.; Richter, S.; Lamppa, G.; Whelan, J.; Glaser, E.
Processing of the dual targeted precursor protein of glutathione reductase in mitochondria and chloroplasts
J. Mol. Biol.
343
639-647
2004
Solanum tuberosum
Manually annotated by BRENDA team
Murcha, M.W.; Elhafez, D.; Millar, A.H.; Whelan, J.
The N-terminal extension of plant mitochondrial carrier proteins is removed by two-step processing: the first cleavage is by the mitochondrial processing peptidase
J. Mol. Biol.
344
443-454
2004
Saccharomyces cerevisiae, Solanum tuberosum, Saccharomyces cerevisiae MY111-2
Manually annotated by BRENDA team
Nishino, T.G.; Kitano, K.; Kojima, K.; Ogishima, T.; Ito, A.; Kitada, S.
Spatial orientation of mitochondrial processing peptidase and a preprotein revealed by fluorescence resonance energy transfer
J. Biochem.
141
889-895
2007
Saccharomyces cerevisiae
Manually annotated by BRENDA team
Mukhopadhyay, A.; Yang, C.S.; Wei, B.; Weiner, H.
Precursor protein is readily degraded in mitochondrial matrix space if the leader is not processed by mitochondrial processing peptidase
J. Biol. Chem.
282
37266-37275
2007
Saccharomyces cerevisiae, Homo sapiens
Manually annotated by BRENDA team
Yoon, T.; Dizin, E.; Cowan, J.A.
N-terminal iron-mediated self-cleavage of human frataxin: regulation of iron binding and complex formation with target proteins
J. Biol. Inorg. Chem.
12
535-542
2007
Homo sapiens
Manually annotated by BRENDA team
Osman, C.; Wilmes, C.; Tatsuta, T.; Langer, T.
Prohibitins interact genetically with Atp23, a novel processing peptidase and chaperone for the F1Fo-ATP synthase
Mol. Biol. Cell
18
627-635
2007
Saccharomyces cerevisiae
Manually annotated by BRENDA team
Burri, L.; Williams, B.A.; Bursac, D.; Lithgow, T.; Keeling, P.J.
Microsporidian mitosomes retain elements of the general mitochondrial targeting system
Proc. Natl. Acad. Sci. USA
103
15916-15920
2006
Antonospora locustae, no activity in Encephalitozoon cuniculi
Manually annotated by BRENDA team
Nagayama, K.; Itono, S.; Yoshida, T.; Ishiguro, S.; Ochiai, H.; Ohmachi, T.
Antisense RNA inhibition of the beta subunit of the Dictyostelium discoideum mitochondrial processing peptidase induces the expression of mitochondrial proteins
Biosci. Biotechnol. Biochem.
72
1836-1846
2008
Dictyostelium discoideum
Manually annotated by BRENDA team
Smid, O.; Matuskova, A.; Harris, S.R.; Kucera, T.; Novotny, M.; Horvathova, L.; Hrdy, I.; Kutejova, E.; Hirt, R.P.; Embley, T.M.; Janata, J.; Tachezy, J.
Reductive evolution of the mitochondrial processing peptidases of the unicellular parasites trichomonas vaginalis and giardia intestinalis
PLoS Pathog.
4
e1000243
2008
Giardia intestinalis, Trichomonas vaginalis
Manually annotated by BRENDA team
Voegtle, F.N.; Wortelkamp, S.; Zahedi, R.P.; Becker, D.; Leidhold, C.; Gevaert, K.; Kellermann, J.; Voos, W.; Sickmann, A.; Pfanner, N.; Meisinger, C.
Global analysis of the mitochondrial N-proteome identifies a processing peptidase critical for protein stability
Cell
139
428-439
2009
Saccharomyces cerevisiae
Manually annotated by BRENDA team
Naamati, A.; Regev-Rudzki, N.; Galperin, S.; Lill, R.; Pines, O.
Dual targeting of Nfs1 and discovery of its novel processing enzyme, Icp55
J. BIOL. CHEM.
284
30200-30208
2009
Saccharomyces cerevisiae
Manually annotated by BRENDA team
Nagayama, K.; Ohmachi, T.
Mitochondrial processing peptidase activity is controlled by the processing of alpha-MPP during development in Dictyostelium discoideum
Microbiology
156
978-989
2010
Dictyostelium discoideum (Q86A84), Dictyostelium discoideum
Manually annotated by BRENDA team
Ohtsuka, J.; Ichihara, Y.; Ebihara, A.; Nagata, K.; Tanokura, M.
Crystal structure of TTHA1264, a putative M16-family zinc peptidase from Thermus thermophilus HB8 that is homologous to the β subunit of mitochondrial processing peptidase
Proteins
75
774-780
2009
Thermus thermophilus (Q5SIV0), Thermus thermophilus HB8 / ATCC 27634 / DSM 579 (Q5SIV0)
Manually annotated by BRENDA team
Mossmann, D.; Meisinger, C.; Voegtle, F.
Processing of mitochondrial presequences
Biochim. Biophys. Acta
1819
1098-1106
2012
Saccharomyces cerevisiae
Manually annotated by BRENDA team
Teixeira, P.; Glaser, E.
Processing peptidases in mitochondria and chloroplasts
Biochim. Biophys. Acta
1833
360-370
2013
Homo sapiens
Manually annotated by BRENDA team
Greene, A.; Grenier, K.; Aguileta, M.; Muise, S.; Farazifard, R.; Haque, M.; McBride, H.; Park, D.; Fon, E.
Mitochondrial processing peptidase regulates PINK1 processing, import and Parkin recruitment
EMBO Rep.
13
378-385
2012
Homo sapiens
Manually annotated by BRENDA team
Endow, J.; Inoue, K.
Stable complex formation of thylakoidal processing peptidase and PGRL1
FEBS Lett.
587
2226-2231
2013
Arabidopsis thaliana
Manually annotated by BRENDA team
Mach, J.; Poliak, P.; Matuskova, A.; Zarsky V.; Janata, J.; Lukes, J.; Tachezy, J.
An advanced system of the mitochondrial processing peptidase and core protein family in Trypanosoma bruceiand multiple origins of the Core I subunit in eukaryotes
Genome Biol. Evol.
5
860-875
2013
Trypanosoma brucei
Manually annotated by BRENDA team
Desy, S.; Schneider, A.; Mani, J.
Trypanosoma brucei has a canonical mitochondrial processing peptidase
Mol. Biochem. Parasitol.
185
161-164
2012
Trypanosoma brucei
Manually annotated by BRENDA team
Kwasniak, M.; Pogorzelec, L.; Migdal, I.; Smakowska, E.; Janska, H.
Proteolytic system of plant mitochondria
Physiol. Plant.
145
187-195
2012
Arabidopsis thaliana
Manually annotated by BRENDA team
Jobling, R.K.; Assoum, M.; Gakh, O.; Blaser, S.; Raiman, J.A.; Mignot, C.; Roze, E.; Duerr, A.; Brice, A.; Levy, N.; Prasad, C.; Paton, T.; Paterson, A.D.; Roslin, N.M.; Marshall, C.R.; Desvignes, J.P.; Roeckel-Trevisiol, N.; Scherer, S.W.; Rouleau, G.A.; Megarbane, A.; Isaya, G.; Delague, V.; Yoon, G.
PMPCA mutations cause abnormal mitochondrial protein processing in patients with non-progressive cerebellar ataxia
Brain
138
1505-1517
2015
Homo sapiens (Q10713 AND O75439), Homo sapiens
Manually annotated by BRENDA team
Joshi, M.; Anselm, I.; Shi, J.; Bale, T.A.; Towne, M.; Schmitz-Abe, K.; Crowley, L.; Giani, F.C.; Kazerounian, S.; Markianos, K.; Lidov, H.G.; Folkerth, R.; Sankaran, V.G.; Agrawal, P.B.
Mutations in the substrate binding glycine-rich loop of the mitochondrial processing peptidase-alpha protein (PMPCA) cause a severe mitochondrial disease
Cold Spring Harb. Mol. Case Stud.
2
a000786
2016
Homo sapiens (Q10713), Homo sapiens
Manually annotated by BRENDA team
Marcondes, M.F.; Alves, F.M.; Assis, D.M.; Hirata, I.Y.; Juliano, L.; Oliveira, V.; Juliano, M.A.
Substrate specificity of mitochondrial intermediate peptidase analysed by a support-bound peptide library
FEBS open bio
5
429-436
2015
Homo sapiens (Q10713 AND O75439)
Manually annotated by BRENDA team
Burkhart, J.M.; Taskin, A.A.; Zahedi, R.P.; Voegtle, F.N.
Quantitative profiling for substrates of the mitochondrial presequence processing protease reveals a set of nonsubstrate proteins increased upon proteotoxic stress
J. Proteome Res.
14
4550-4563
2015
Saccharomyces cerevisiae (P10507 AND P11914), Saccharomyces cerevisiae ATCC 204508 (P10507 AND P11914)
Manually annotated by BRENDA team
Woellhaf, M.W.; Sommer, F.; Schroda, M.; Herrmann, J.M.
Proteomic profiling of the mitochondrial ribosome identifies Atp25 as a composite mitochondrial precursor protein
Mol. Biol. Cell
27
3031-3039
2016
Saccharomyces cerevisiae
Manually annotated by BRENDA team