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The mitochondrial TIM22 preprotein translocase is highly conserved throughout the eukaryotic kingdom.

Abstract
The Mohr-Tranebjaerg syndrome (MTS), a neurodegenerative syndrome characterized by progressive sensorineural hearing loss, dystonia, mental retardation and blindness, is a mitochondrial disease caused by mutations in the deafness/dystonia peptide 1 (DDP1) gene. DDP1 shows similarity to the yeast proteins Tim9, Tim10 and Tim12, components of the mitochondrial import machinery for carrier proteins. Here, we show that DDP1 belongs to a large family of evolutionarily conserved proteins. We report the identification, chromosomal localization and expressional analysis of six human family members which represent further candidate genes for neurodegenerative diseases.
AuthorsM F Bauer, U Rothbauer, N Mühlenbein, R J Smith, K Gerbitz, W Neupert, M Brunner, S Hofmann
JournalFEBS letters (FEBS Lett) Vol. 464 Issue 1-2 Pg. 41-7 (Dec 24 1999) ISSN: 0014-5793 [Print] England
PMID10611480 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Carrier Proteins
  • DDP2 protein, E coli
  • DNA, Complementary
  • DNA-Binding Proteins
  • Escherichia coli Proteins
  • Fungal Proteins
  • Membrane Proteins
  • Membrane Transport Proteins
  • Mitochondrial Membrane Transport Proteins
  • Mitochondrial Precursor Protein Import Complex Proteins
  • Mitochondrial Proteins
  • Protein Precursors
  • Saccharomyces cerevisiae Proteins
  • TIM10 protein, S cerevisiae
  • TIM13 protein, S cerevisiae
  • TIM22 protein, S cerevisiae
  • TIMM13 protein, human
  • TIMM8A protein, human
  • Tim9 protein, S cerevisiae
Topics
  • Amino Acid Sequence
  • Blotting, Northern
  • Carrier Proteins (chemistry, genetics, metabolism)
  • Conserved Sequence
  • DNA, Complementary (metabolism)
  • DNA-Binding Proteins (chemistry, metabolism)
  • Escherichia coli Proteins
  • Evolution, Molecular
  • Fungal Proteins (chemistry, metabolism)
  • Humans
  • Membrane Proteins (chemistry, genetics, metabolism)
  • Membrane Transport Proteins
  • Mitochondria (enzymology)
  • Mitochondrial Membrane Transport Proteins
  • Mitochondrial Precursor Protein Import Complex Proteins
  • Mitochondrial Proteins
  • Molecular Sequence Data
  • Multigene Family
  • Phylogeny
  • Physical Chromosome Mapping
  • Protein Precursors (chemistry, genetics, metabolism)
  • Saccharomyces cerevisiae (enzymology)
  • Saccharomyces cerevisiae Proteins
  • Sequence Homology, Amino Acid
  • Tissue Distribution

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