title: TIM29 is a subunit of the human carrier translocase required for protein transport.
abstract: Hydrophobic inner mitochondrial membrane proteins with internal targeting signals, such as the metabolite carriers, use the carrier translocase (TIM22 complex) for transport into the inner membrane. Defects in this transport pathway have been associated with neurodegenerative disorders. While the TIM22 complex is well studied in baker's yeast, very little is known about the mammalian TIM22 complex. Using immunoprecipitation, we purified the human carrier translocase and identified a mitochondrial inner membrane protein TIM29 as a novel component, specific to metazoa. We show that TIM29 is a constituent of the 440 kDa TIM22 complex and interacts with oxidized TIM22. Our analyses demonstrate that TIM29 is required for the structural integrity of the TIM22 complex and for import of substrate proteins by the carrier translocase.
authors.affiliation: Department of Cellular Biochemistry, University Medical Center Göttingen, Germany.
authors.forename: Sylvie
authors.initials: S
authors.lastname: Callegari
authors.affiliation: Department of Cellular Biochemistry, University Medical Center Göttingen, Germany.
authors.forename: Frank
authors.initials: F
authors.lastname: Richter
authors.affiliation: International Institute of Molecular and Cell Biology, Warsaw, Poland.
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Chojnacka
authors.affiliation: Department of NanoBiophotonics, Mitochondrial Structure and Dynamics Group, Max Planck Institute for Biophysical Chemistry, Göttingen, Germany.|Department of Neurology, University Medical Center Göttingen, Germany.
authors.forename: Daniel C
authors.initials: DC
authors.lastname: Jans
authors.affiliation: Department of Cellular Biochemistry, University Medical Center Göttingen, Germany.
authors.forename: Isotta
authors.initials: I
authors.lastname: Lorenzi
authors.affiliation: Department of Cellular Biochemistry, University Medical Center Göttingen, Germany.
authors.forename: David
authors.initials: D
authors.lastname: Pacheu-Grau
authors.affiliation: Department of NanoBiophotonics, Mitochondrial Structure and Dynamics Group, Max Planck Institute for Biophysical Chemistry, Göttingen, Germany.|Department of Neurology, University Medical Center Göttingen, Germany.
authors.forename: Stefan
authors.initials: S
authors.lastname: Jakobs
authors.affiliation: Bioanalytical Mass Spectrometry Group, Max Planck Institute for Biophysical Chemistry, Göttingen, Germany.|Bioanalytics, Institute for Clinical Chemistry, University Medical Center Göttingen, Germany.
authors.forename: Christof
authors.initials: C
authors.lastname: Lenz
authors.affiliation: Bioanalytical Mass Spectrometry Group, Max Planck Institute for Biophysical Chemistry, Göttingen, Germany.|Bioanalytics, Institute for Clinical Chemistry, University Medical Center Göttingen, Germany.
authors.forename: Henning
authors.initials: H
authors.lastname: Urlaub
authors.affiliation: Department of Cellular Biochemistry, University Medical Center Göttingen, Germany.
authors.forename: Jan
authors.initials: J
authors.lastname: Dudek
authors.affiliation: International Institute of Molecular and Cell Biology, Warsaw, Poland.
authors.forename: Agnieszka
authors.initials: A
authors.lastname: Chacinska
authors.affiliation: Department of Cellular Biochemistry, University Medical Center Göttingen, Germany.|MaxPlanck Institute for Biophysical Chemistry, Göttingen, Germany.
authors.forename: Peter
authors.initials: P
authors.lastname: Rehling
mesh_terms: D000595:Amino Acid Sequence; D057809:HEK293 Cells; D006367:HeLa Cells; D006801:Humans; D008928:Mitochondria / Q000378:metabolism; D033681:Mitochondrial Membrane Transport Proteins / Q000737:chemistry / Q000378:metabolism*; D000090622:Mitochondrial Precursor Protein Import Complex Proteins; D010084:Oxidation-Reduction; D021381:Protein Transport
chemical_list: D033681:Mitochondrial Membrane Transport Proteins; D000090622:Mitochondrial Precursor Protein Import Complex Proteins; C000618451:TIMM29 protein, human; C489971:TOMM22 protein, human
keywords: TIM22; TIM29; carrier translocase; membrane protein; mitochondria; protein translocation