Literature for peptidase M03.006: mitochondrial intermediate peptidase

Summary Gene structure Alignment Tree Sequences Sequence features Distribution Literature Substrates

(Topics flags: A Assay, M Mutation, I Inhibitor, P Specificity, E Expression, V Review. To select only the references relevant to a single topic, click the link above. See explanation.)

2025

Ishimatsu,M., Taki,K., Hayami,A., Kato,K., Nozaki,Y., Mizunoe,Y., Narita,T., Mori,R., Higami,Y. and Kobayashi,M. Co-overexpression of the caloric restriction-induced mitochondrial factors PGC-1alpha and MIPEP upregulates Phospho1 expression in adipocytes FEBS Open Bio (2025) PubMed Europe PubMed DOI
Ludwig,K. and Heidorn-Czarna,M. Mitochondrial Proteases and Their Roles in Mitophagy in Plants, Animals, and Yeast Plant Cell Physiol (2025) PubMed Europe PubMed DOI V
Nozaki,Y., Kobayashi,M., Fukuoh,T., Ishimatsu,M., Narita,T., Taki,K., Hirao,Y., Ayabe,S., Yokoyama,M., Otani,Y., Mizunoe,Y., Matsumoto,M., Ohno,N., Kaifu,T., Okazaki,S., Goitsuka,R., Nakagawa,Y., Shimano,H., Iwakura,Y. and Higami,Y. Mipep deficiency in adipocytes impairs mitochondrial protein maturation and leads to systemic inflammation and metabolic dysfunctions Sci Rep (2025) 15, 12839-12839. PubMed Europe PubMed DOI PMC EPMC

2022

Heidorn-Czarna,M., Maziak,A. and Janska,H. Protein Processing in Plant Mitochondria Compared to Yeast and Mammals Front Plant Sci (2022) 13, 824080-824080. PubMed Europe PubMed DOI PMC EPMC V
Wachoski-Dark,E., Zhao,T., Khan,A., Shutt,T.E. and Greenway,S.C. Mitochondrial Protein Homeostasis and Cardiomyopathy Int J Mol Sci (2022) 23 PubMed Europe PubMed DOI PMC EPMC V
Wang,L., Lu,P., Yin,J., Xu,K., Xiang,D., Zhang,Z., Zhang,H., Zheng,B., Zhou,W., Wang,C. and Yang,S. Case report: Rare novel MIPEP compound heterozygous variants presenting with hypertrophic cardiomyopathy, severe lactic acidosis and hypotonia in a Chinese infant Front Cardiovasc Med (2022) 9, 1095882-1095882. PubMed Europe PubMed DOI PMC EPMC

2021

Pulman,J., Ruzzenente,B., Horak,M., Barcia,G., Boddaert,N., Munnich,A., Rotig,A. and Metodiev,M.D. Variants in the MIPEP gene presenting with complex neurological phenotype without cardiomyopathy, impair OXPHOS protein maturation and lead to a reduced OXPHOS abundance in patient cells Mol Genet Metab (2021) 134, 267-273. PubMed Europe PubMed DOI
Sim,J., Park,J., Woo,H.A. and Rhee,S.G. Maturation of Mitochondrially Targeted Prx V Involves a Second Cleavage by Mitochondrial Intermediate Peptidase That Is Sensitive to Inhibition by H2O2 Antioxidants (Basel) (2021) 10 PubMed Europe PubMed DOI PMC EPMC

2020

Jacques,S., van der Sloot,A.M., C Huard,C., Coulombe-Huntington,J., Tsao,S., Tollis,S., Bertomeu,T., Culp,E.J., Pallant,D., Cook,M.A., Bonneil,E., Thibault,P., Wright,G.D. and Tyers,M. Imipridone anticancer compounds ectopically activate the ClpP protease and represent a new scaffold for antibiotic development Genetics (2020) 214, 1103-1120. PubMed Europe PubMed DOI

2017

Gomes,F., Palma,F.R., Barros,M.H., Tsuchida,E.T., Turano,H.G., Alegria,T.G.P., Demasi,M. and Netto,L.E.S. Proteolytic cleavage by the inner membrane peptidase (IMP) complex or Oct1 peptidase controls the localization of the yeast peroxiredoxin Prx1 to distinct mitochondrial compartments J Biol Chem (2017) 292, 17011-17024. PubMed Europe PubMed DOI
Kobayashi,M., Takeda,K., Narita,T., Nagai,K., Okita,N., Sudo,Y., Miura,Y., Tsumoto,H., Nakagawa,Y., Shimano,H. and Higami,Y. Mitochondrial intermediate peptidase is a novel regulator of sirtuin-3 activation by caloric restriction FEBS Lett (2017) 591, 4067-4073. PubMed Europe PubMed DOI
Veling,M.T., Reidenbach,A.G., Freiberger,E.C., Kwiecien,N.W., Hutchins,P.D., Drahnak,M.J., Jochem,A., Ulbrich,A., Rush,M.J.P., Russell,J.D., Coon,J.J. and Pagliarini,D.J. Multi-omic mitoprotease profiling defines a role for Oct1p in coenzyme Q production Mol Cell (2017) 68, 970-977. PubMed Europe PubMed DOI PMC EPMC

2016

Eldomery,M.K., Akdemir,Z.C., Vogtle,F.N., Charng,W.L., Mulica,P., Rosenfeld,J.A., Gambin,T., Gu,S., Burrage,L.C., Al Shamsi,A., Penney,S., Jhangiani,S.N., Zimmerman,H.H., Muzny,D.M., Wang,X., Tang,J., Medikonda,R., Ramachandran,P.V., Wong,L.J., Boerwinkle,E., Gibbs,R.A., Eng,C.M., Lalani,S.R., Hertecant,J., Rodenburg,R.J., Abdul-Rahman,O.A., Yang,Y., Xia,F., Wang,M.C., Lupski,J.R., Meisinger,C. and Sutton,V.R. MIPEP recessive variants cause a syndrome of left ventricular non-compaction, hypotonia, and infantile death Genome Med (2016) 8, 106-106. PubMed Europe PubMed DOI PMC EPMC M
Kmiec,B., Teixeira,P.F., Murcha,M.W. and Glaser,E. Divergent evolution of the M3A family of metallopeptidases in plants Physiol Plant (2016) 157, 380-388. PubMed Europe PubMed DOI

2015

Carrie,C., Venne,A.S., Zahedi,R.P. and Soll,J. Identification of cleavage sites and substrate proteins for two mitochondrial intermediate peptidases in Arabidopsis thaliana J Exp Bot (2015) 66, 2691-2708. PubMed Europe PubMed DOI P
Marcondes,M.F., Alves,F.M., Assis,D.M., Hirata,I.Y., Juliano,L., Oliveira,V. and Juliano,M.A. Substrate specificity of mitochondrial intermediate peptidase analysed by a support-bound peptide library FEBS Open Bio (2015) 5, 429-436. PubMed Europe PubMed DOI PMC EPMC P

2013

Teixeira,P.F. and Glaser,E. Processing peptidases in mitochondria and chloroplasts Biochim Biophys Acta (2013) 1833, 360-370. PubMed Europe PubMed DOI V

2012

Chen,B.Z., Gui,F., Xie,B.G., Zou,F., Jiang,Y.J. and Deng,Y.J. Sequence and comparative analysis of the MIP gene in Chinese straw mushroom, Volvariella volvacea Genome (2012) 55, 667-672. PubMed Europe PubMed DOI
Gakh,O. and Isaya,G. Mitochondrial intermediate peptidase [ISSN:978-0-12-407744-7] (2012) 3, 524-529. DOI
Mossmann,D., Meisinger,C. and Vogtle,F.N. Processing of mitochondrial presequences Biochim Biophys Acta (2012) 1819, 1098-1106. PubMed Europe PubMed DOI V
Vaidya,M. and Panchal,H. In silico investigation and structural characterization of virulent factor and a metallo peptidase present in Helicobacter pylori strain J99 Interdiscip Sci (2012) 4, 302-309. PubMed Europe PubMed DOI

2011

Lee,S.F., Srinivasan,B., Sephton,C.F., Dries,D.R., Wang,B., Yu,C., Wang,Y., Dewey,C.M., Shah,S., Jiang,J. and Yu,G. Gamma-secretase-regulated proteolysis of the Notch receptor by mitochondrial intermediate peptidase J Biol Chem (2011) 286, 27447-27453. PubMed Europe PubMed DOI
Vogtle,F.N., Prinz,C., Kellermann,J., Lottspeich,F., Pfanner,N. and Meisinger,C. Mitochondrial protein turnover: role of the precursor intermediate peptidase Oct1 in protein stabilization Mol Biol Cell (2011) 22, 2135-2143. PubMed Europe PubMed DOI

2010

Marcondes,M.F., Torquato,R.J., Assis,D.M., Juliano,M.A., Hayashi,M.A. and Oliveira,V. Mitochondrial intermediate peptidase: expression in Escherichia coli and improvement of its enzymatic activity detection with FRET substrates Biochem Biophys Res Commun (2010) 391, 123-128. PubMed Europe PubMed DOI A E I

2008

van der Nest,M.A., Slippers,B., Stenlid,J., Wilken,P.M., Vasaitis,R., Wingfield,M.J. and Wingfield,B.D. Characterization of the systems governing sexual and self-recognition in the white rot homobasidiomycete Amylostereum areolatum Curr Genet (2008) 53, 323-336. PubMed Europe PubMed DOI

2004

Isaya,G. Mitochondrial intermediate peptidase [ISSN:0-12-079610-4] (2004) 2, 366-369. V
James,T.Y., Kues,U., Rehner,S.A. and Vilgalys,R. Evolution of the gene encoding mitochondrial intermediate peptidase and its cosegregation with the A mating-type locus of mushroom fungi Fungal Genet Biol (2004) 41, 381-390. PubMed Europe PubMed DOI

2002

[YEAR:2-9-2002]Gakh,O., Cavadini,P. and Isaya,G. Mitochondrial processing peptidases Biochim Biophys Acta (2-9-2002) 1592, 63-77. PubMed Europe PubMed DOI

2000

[YEAR:15-4-2000]Chew,A., Sirugo,G., Alsobrook,J.P. and Isaya,G. Functional and genomic analysis of the human mitochondrial intermediate peptidase, a putative protein partner of frataxin Genomics (15-4-2000) 65, 104-112. PubMed Europe PubMed DOI

1999

Branda,S.S., Yang,Z.Y., Chew,A. and Isaya,G. Mitochondrial intermediate peptidase and the yeast frataxin homolog together maintain mitochondrial iron homeostasis in Saccharomyces cerevisiae Hum Mol Genet (1999) 8, 1099-1110. PubMed Europe PubMed DOI
Seibel,M., Bachmann,C., Schmiedel,J., Wilken,N., Wilde,F., Reichmann,H., Isaya,G., Seibel,P. and Pfeiler,D. Processing of artificial peptide-DNA-conjugates by the mitochondrial intermediate peptidase (MIP) Biol Chem (1999) 380, 961-967. PubMed Europe PubMed DOI

1997

Chew,A., Buck,E.A., Peretz,S., Sirugo,G., Rinaldo,P. and Isaya,G. Cloning, expression, and chromosomal assignment of the human mitochondrial intermediate peptidase gene (MIPEP) Genomics (1997) 40, 493-496. PubMed Europe PubMed DOI
Isaya,G., Branda,S.S. and Chew,A. Mitochondrial intermediate peptidase: natural substrates and biologic role [ISSN:ISBN 90 5199 322 6 (IOS Press)] (1997) , 340-347.
Nett,J.H., Denke,E. and Trumpower,B.L. Two-step processing is not essential for the import and assembly of functionally active iron-sulfur protein into the cytochrome bc1 complex in Saccharomyces cerevisiae J Biol Chem (1997) 272, 2212-2217. PubMed Europe PubMed DOI

1996

Chew,A., Rollins,R.A., Sakati,W.R. and Isaya,G. Mutations in a putative zinc-binding domain inactivate the mitochondrial intermediate peptidase Biochem Biophys Res Commun (1996) 226, 822-829. PubMed Europe PubMed DOI

1995

Branda,S.S. and Isaya,G. Prediction and identification of new natural substrates of the yeast mitochondrial intermediate peptidase J Biol Chem (1995) 270, 27366-27373. PubMed Europe PubMed DOI
Isaya,G., Sakati,W.R., Rollins,R.A., Shen,G.P., Hanson,L.C., Ullrich,R.C. and Novotny,C.P. Mammalian mitochondrial intermediate peptidase: structure/function analysis of a new homologue from Schizophyllum commune and relationship to thimet oligopeptidases Genomics (1995) 28, 450-461. PubMed Europe PubMed DOI
Isaya,G. and Kalousek,F. Mitochondrial intermediate peptidase Methods Enzymol (1995) 248, 556-567. PubMed Europe PubMed DOI V

1994

Isaya,G., Miklos,D. and Rollins,R.A. MIP1, a new yeast gene homologous to the rat mitochondrial intermediate peptidase gene, is required for oxidative metabolism in Saccharomyces cerevisiae Mol Cell Biol (1994) 14, 5603-5616. PubMed Europe PubMed PMC EPMC

1992

Isaya,G., Kalousek,F. and Rosenberg,L.E. Amino-terminal octapeptides function as recognition signals for the mitochondrial intermediate peptidase J Biol Chem (1992) 267, 7904-7910. PubMed Europe PubMed
Isaya,G., Kalousek,F. and Rosenberg,L.E. Sequence analysis of rat mitochondrial intermediate peptidase: similarity to zinc metallopeptidases and to a putative yeast homologue Proc Natl Acad Sci U S A (1992) 89, 8317-8321. PubMed Europe PubMed PMC EPMC
Kalousek,F., Isaya,G. and Rosenberg,L.E. Rat liver mitochondrial intermediate peptidase (MIP): Purification and initial characterization EMBO J (1992) 11, 2803-2809. PubMed Europe PubMed PMC EPMC

1989

Hendrick,J.P., Hodges,P.E. and Rosenberg,L.E. Survey of amino-terminal proteolytic cleavage sites in mitochondrial precursor proteins: leader peptides cleaved by two matrix proteases share a three-amino acid motif Proc Natl Acad Sci U S A (1989) 86, 4056-4060. PubMed Europe PubMed PMC EPMC

1988

Kalousek,F., Hendrick,J.P. and Rosenberg,L.E. Two mitochondrial matrix proteases act sequentially in the processing of mammalian matrix enzymes Proc Natl Acad Sci U S A (1988) 85, 7536-7540. PubMed Europe PubMed PMC EPMC

1987

Sztul,E.S., Hendrick,J.P., Kraus,J.P., Wall,D., Kalousek,F. and Rosenberg,L.E. Import of rat ornithine transcarbamylase precursor into mitochondria: two-step processing of the leader peptide J Cell Biol (1987) 105, 2631-2639. PubMed Europe PubMed PMC EPMC

1985

Hurt,E.C., Pesold-Hurt,B., Suda,K., Oppliger,W. and Schatz,G. The first twelve amino acids (less than half of the pre-sequence) of an imported mitochondrial protein can direct mouse cytosolic dihydrofolate reductase into the yeast mitochondrial matrix EMBO J (1985) 4, 2061-2068. PubMed Europe PubMed PMC EPMC