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

FEBS Lett. 1999 Dec 24;464(1-2):41-7. doi: 10.1016/s0014-5793(99)01665-8.

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amino Acid Sequence
  • Blotting, Northern
  • Carrier Proteins / chemistry*
  • Carrier Proteins / genetics
  • Carrier Proteins / metabolism
  • Conserved Sequence
  • DNA, Complementary / metabolism
  • DNA-Binding Proteins / chemistry
  • DNA-Binding Proteins / metabolism
  • Escherichia coli Proteins*
  • Evolution, Molecular
  • Fungal Proteins / chemistry
  • Fungal Proteins / metabolism
  • Humans
  • Membrane Proteins / chemistry*
  • Membrane Proteins / genetics
  • Membrane Proteins / 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*
  • Protein Precursors / genetics
  • Protein Precursors / metabolism
  • Saccharomyces cerevisiae / enzymology
  • Saccharomyces cerevisiae Proteins*
  • Sequence Homology, Amino Acid
  • Tissue Distribution

Substances

  • 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

Associated data

  • GENBANK/AF223950
  • GENBANK/AF223951