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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)
 

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Gene Review: 

MTM1  -  Mtm1p

Saccharomyces cerevisiae S288c

Synonyms: G9175, Manganese trafficking factor for mitochondrial SOD2, Mitochondrial carrier protein MTM1, YGR257C
 
 
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Disease relevance of MTM1

 

High impact information on MTM1

  • The bulk of mitochondrial iron is normally unavailable to SOD2, but when mitochondrial iron homeostasis is disrupted, for example, by mutations in S. cerevisiae mtm1, ssq1 and grx5, iron accumulates in a reactive form that potently competes with manganese for binding to SOD2, inactivating the enzyme [2].
  • Our studies suggest a connection with SOD2, and we have named the yeast gene MTM1 for manganese trafficking factor for mitochondrial SOD2 [3].
  • Surprisingly, the mitochondrial organelle of mtm1 Delta mutants shows no deficiency in manganese levels [3].

References

  1. Genetic analysis of the myotubularin family of phosphatases in Caenorhabditis elegans. Xue, Y., Fares, H., Grant, B., Li, Z., Rose, A.M., Clark, S.G., Skolnik, E.Y. J. Biol. Chem. (2003) [Pubmed]
  2. The effects of mitochondrial iron homeostasis on cofactor specificity of superoxide dismutase 2. Yang, M., Cobine, P.A., Molik, S., Naranuntarat, A., Lill, R., Winge, D.R., Culotta, V.C. EMBO J. (2006) [Pubmed]
  3. Manganese activation of superoxide dismutase 2 in Saccharomyces cerevisiae requires MTM1, a member of the mitochondrial carrier family. Luk, E., Carroll, M., Baker, M., Culotta, V.C. Proc. Natl. Acad. Sci. U.S.A. (2003) [Pubmed]
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