title: Requirement for the yeast gene LON in intramitochondrial proteolysis and maintenance of respiration.
abstract: The role of protein degradation in mitochondrial homeostasis was explored by cloning of a gene from Saccharomyces cerevisiae that encodes a protein resembling the adenosine triphosphate (ATP)-dependent bacterial protease Lon. The predicted yeast protein has a typical mitochondrial matrix-targeting sequence at its amino terminus. Yeast cells lacking a functional LON gene contained a nonfunctional mitochondrial genome, were respiratory-deficient, and lacked an ATP-dependent proteolytic activity present in the mitochondria of Lon+ cells. 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.
authors.affiliation: Biozentrum der Universität Basel, Switzerland.
authors.forename: C K
authors.initials: CK
authors.lastname: Suzuki
authors.forename: K
authors.initials: K
authors.lastname: Suda
authors.forename: N
authors.initials: N
authors.lastname: Wang
authors.forename: G
authors.initials: G
authors.lastname: Schatz
mesh_terms: D049069:ATP-Dependent Proteases; D000251:Adenosine Triphosphatases / Q000235:genetics / Q000378:metabolism; D000595:Amino Acid Sequence; D001483:Base Sequence; D003001:Cloning, Molecular; D005656:Fungal Proteins / Q000378:metabolism; D005800:Genes, Fungal; D006360:Heat-Shock Proteins / Q000235:genetics* / Q000378:metabolism; D008854:Microscopy, Electron; D008928:Mitochondria / Q000378:metabolism* / Q000648:ultrastructure; D008969:Molecular Sequence Data; D010101:Oxygen Consumption; D012441:Saccharomyces cerevisiae / Q000235:genetics* / Q000378:metabolism; D012697:Serine Endopeptidases / Q000235:genetics* / Q000378:metabolism
chemical_list: D005656:Fungal Proteins; D006360:Heat-Shock Proteins; D049069:ATP-Dependent Proteases; D012697:Serine Endopeptidases; D000251:Adenosine Triphosphatases