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
  1. Ludwig,K. and Heidorn-Czarna,M.
    Mitochondrial Proteases and Their Roles in Mitophagy in Plants, Animals, and Yeast
    Plant Cell Physiol PubMed  Europe PubMed DOI  V
  2. 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 Rep15, 12839-12839. PubMed  Europe PubMed DOI
  3. 2022
  4. Heidorn-Czarna,M., Maziak,A. and Janska,H.
    Protein Processing in Plant Mitochondria Compared to Yeast and Mammals
    Front Plant Sci13, 824080-824080. PubMed  Europe PubMed DOI  V
  5. Wachoski-Dark,E., Zhao,T., Khan,A., Shutt,T.E. and Greenway,S.C.
    Mitochondrial Protein Homeostasis and Cardiomyopathy
    Int J Mol Sci23, PubMed  Europe PubMed DOI  V
  6. 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 Med9, 1095882-1095882. PubMed  Europe PubMed DOI
  7. 2021
  8. 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 Metab134, 267-273. PubMed  Europe PubMed DOI
  9. 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)10, PubMed  Europe PubMed DOI
  10. 2020
  11. 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
    Genetics214, 1103-1120. PubMed  Europe PubMed DOI
  12. 2017
  13. 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 Chem292, 17011-17024. PubMed  Europe PubMed DOI
  14. 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 Lett591, 4067-4073. PubMed  Europe PubMed DOI
  15. 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 Cell68, 970-977. PubMed  Europe PubMed DOI
  16. 2016
  17. 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 Med8, 106-106. PubMed  Europe PubMed DOI  M
  18. Kmiec,B., Teixeira,P.F., Murcha,M.W. and Glaser,E.
    Divergent evolution of the M3A family of metallopeptidases in plants
    Physiol Plant157, 380-388. PubMed  Europe PubMed DOI
  19. 2015
  20. 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 Bot66, 2691-2708. PubMed  Europe PubMed DOI  P
  21. 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 Bio5, 429-436. PubMed  Europe PubMed DOI  P
  22. 2013
  23. Teixeira,P.F. and Glaser,E.
    Processing peptidases in mitochondria and chloroplasts
    Biochim Biophys Acta1833, 360-370. PubMed  Europe PubMed DOI  V
  24. 2012
  25. 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
    Genome55, 667-672. PubMed  Europe PubMed DOI
  26. Gakh,O. and Isaya,G.
    Mitochondrial intermediate peptidase
    [ISSN:978-0-12-407744-7]3, 524-529. DOI
  27. Mossmann,D., Meisinger,C. and Vogtle,F.N.
    Processing of mitochondrial presequences
    Biochim Biophys Acta1819, 1098-1106. PubMed  Europe PubMed DOI  V
  28. 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 Sci4, 302-309. PubMed  Europe PubMed DOI
  29. 2011
  30. 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 Chem286, 27447-27453. PubMed  Europe PubMed DOI
  31. 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 Cell22, 2135-2143. PubMed  Europe PubMed DOI
  32. 2010
  33. 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 Commun391, 123-128. PubMed  Europe PubMed DOI  A  E  I
  34. 2008
  35. 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 Genet53, 323-336. PubMed  Europe PubMed DOI
  36. 2004
  37. Isaya,G.
    Mitochondrial intermediate peptidase
    [ISSN:0-12-079610-4]2, 366-369.  V
  38. 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 Biol41, 381-390. PubMed  Europe PubMed DOI
  39. 2002
  40. [YEAR:2-9-2002]Gakh,O., Cavadini,P. and Isaya,G.
    Mitochondrial processing peptidases
    Biochim Biophys Acta1592, 63-77. PubMed  Europe PubMed DOI
  41. 2000
  42. [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
    Genomics65, 104-112. PubMed  Europe PubMed DOI
  43. 1999
  44. 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 Genet8, 1099-1110. PubMed  Europe PubMed DOI
  45. 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 Chem380, 961-967. PubMed  Europe PubMed DOI
  46. 1997
  47. 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)
    Genomics40, 493-496. PubMed  Europe PubMed DOI
  48. Isaya,G., Branda,S.S. and Chew,A.
    Mitochondrial intermediate peptidase: natural substrates and biologic role
    [ISSN:ISBN 90 5199 322 6 (IOS Press)]340-347.
  49. 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 Chem272, 2212-2217. PubMed  Europe PubMed DOI
  50. 1996
  51. 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 Commun226, 822-829. PubMed  Europe PubMed DOI
  52. 1995
  53. Branda,S.S. and Isaya,G.
    Prediction and identification of new natural substrates of the yeast mitochondrial intermediate peptidase
    J Biol Chem270, 27366-27373. PubMed  Europe PubMed DOI
  54. Isaya,G. and Kalousek,F.
    Mitochondrial intermediate peptidase
    Methods Enzymol248, 556-567. PubMed  Europe PubMed DOI  V
  55. 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
    Genomics28, 450-461. PubMed  Europe PubMed DOI
  56. 1994
  57. 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 Biol14, 5603-5616. PubMed  Europe PubMed
  58. 1992
  59. 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 A89, 8317-8321. PubMed  Europe PubMed
  60. Isaya,G., Kalousek,F. and Rosenberg,L.E.
    Amino-terminal octapeptides function as recognition signals for the mitochondrial intermediate peptidase
    J Biol Chem267, 7904-7910. PubMed  Europe PubMed
  61. Kalousek,F., Isaya,G. and Rosenberg,L.E.
    Rat liver mitochondrial intermediate peptidase (MIP): Purification and initial characterization
    EMBO J11, 2803-2809. PubMed  Europe PubMed
  62. 1989
  63. 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 A86, 4056-4060. PubMed  Europe PubMed
  64. 1988
  65. 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 A85, 7536-7540. PubMed  Europe PubMed
  66. 1987
  67. 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 Biol105, 2631-2639. PubMed  Europe PubMed
  68. 1985
  69. 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 J4, 2061-2068. PubMed  Europe PubMed