Gene Symbol: PIM1
Description: ATP-dependent Lon protease PIM1
Alias: ATP-dependent Lon protease PIM1
Species: Saccharomyces cerevisiae S288c

Top Publications

  1. Bohovych I, Donaldson G, Christianson S, Zahayko N, Khalimonchuk O. Stress-triggered activation of the metalloprotease Oma1 involves its C-terminal region and is important for mitochondrial stress protection in yeast. J Biol Chem. 2014;289:13259-72 pubmed publisher
    ..These findings indicate that yeast Oma1 is an important player in IM protein homeostasis and integrity by acting in concert with other intramitochondrial quality control components. ..
  2. Rep M, Nooy J, Guelin E, Grivell L. Three genes for mitochondrial proteins suppress null-mutations in both Afg3 and Rca1 when over-expressed. Curr Genet. 1996;30:206-11 pubmed
    ..growth on non-fermentable carbon sources, all of which affect the biogenesis of respiratory competent mitochondria: PIM1(LON) encodes a matrix-localized ATP-dependent protease involved in the turnover of matrix proteins; OXA1(PET1402) ..
  3. van Dyck L, Neupert W, Langer T. The ATP-dependent PIM1 protease is required for the expression of intron-containing genes in mitochondria. Genes Dev. 1998;12:1515-24 pubmed
    The ATP-dependent PIM1 protease, a Lon-like protease localized in the mitochondrial matrix, is required for mitochondrial genome integrity in yeast...
  4. Wagner I, Arlt H, van Dyck L, Langer T, Neupert W. Molecular chaperones cooperate with PIM1 protease in the degradation of misfolded proteins in mitochondria. EMBO J. 1994;13:5135-45 pubmed
    ATP dependent proteolytic degradation of misfolded proteins in the mitochondrial matrix is mediated by the PIM1 protease and depends on the molecular chaperone proteins mt-hsp70 and Mdj1p...
  5. Suzuki C, Suda K, Wang N, Schatz G. Requirement for the yeast gene LON in intramitochondrial proteolysis and maintenance of respiration. Science. 1994;264:273-6 pubmed
    ..Lon- cells were also impaired in their ability to catalyze the energy-dependent degradation of several mitochondrial matrix proteins and they accumulated electron-dense inclusions in their mitochondrial matrix. ..
  6. Rep M, van Dijl J, Suda K, Schatz G, Grivell L, Suzuki C. Promotion of mitochondrial membrane complex assembly by a proteolytically inactive yeast Lon. Science. 1996;274:103-6 pubmed
    ..It is suggested that the mitochondrial proteases Lon, Afg3p, and Rca1p can also serve a chaperone-like function in the assembly of mitochondrial protein complexes. ..
  7. van Dyck L, Pearce D, Sherman F. PIM1 encodes a mitochondrial ATP-dependent protease that is required for mitochondrial function in the yeast Saccharomyces cerevisiae. J Biol Chem. 1994;269:238-42 pubmed
    The PIM1 nuclear gene in the yeast Saccharomyces cerevisiae encodes a mitochondrial ATP-dependent protease that exhibits over 30% identity with ATP-dependent protease La from Escherichia coli, Lon from Bacillus brevis, and one from ..
  8. Erjavec N, Bayot A, Gareil M, Camougrand N, Nystrom T, Friguet B, et al. Deletion of the mitochondrial Pim1/Lon protease in yeast results in accelerated aging and impairment of the proteasome. Free Radic Biol Med. 2013;56:9-16 pubmed publisher
    ..This accelerated aging of pim1? cells is accompanied by elevated cytosolic levels of oxidized and aggregated proteins, as well as reduced ..
  9. von Janowsky B, Knapp K, Major T, Krayl M, Guiard B, Voos W. Structural properties of substrate proteins determine their proteolysis by the mitochondrial AAA+ protease Pim1. Biol Chem. 2005;386:1307-17 pubmed
    The protease Pim1/LON, a member of the AAA+ family of homo-oligomeric ATP-dependent proteases, is responsible for the degradation of soluble proteins in the mitochondrial matrix...

More Information


  1. Bayot A, Gareil M, Rogowska Wrzesinska A, Roepstorff P, Friguet B, Bulteau A. Identification of novel oxidized protein substrates and physiological partners of the mitochondrial ATP-dependent Lon-like protease Pim1. J Biol Chem. 2010;285:11445-57 pubmed publisher
    ..Among these proteolytic systems, Pim1, a Lon-like serine protease in Saccharomyces cerevisiae, is involved in the control of selective protein turnover ..
  2. Major T, von Janowsky B, Ruppert T, Mogk A, Voos W. Proteomic analysis of mitochondrial protein turnover: identification of novel substrate proteins of the matrix protease pim1. Mol Cell Biol. 2006;26:762-76 pubmed
    ..The protein Pim1/LON has been shown to be the main protease in the mitochondrial matrix responsible for the removal of damaged or ..
  3. Wu X, Li L, Jiang H. Doa1 targets ubiquitinated substrates for mitochondria-associated degradation. J Cell Biol. 2016;213:49-63 pubmed publisher
    ..Collectively, our results demonstrate the essential role of the Doa1-Cdc48(-Ufd1-Npl4)complex in mitochondrial proteostasis and suggest that Doa1 plays dual roles on the Cdc48 complex. ..