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Gene Review

MLH3  -  mismatch repair protein MLH3

Saccharomyces cerevisiae S288c

Synonyms: DNA mismatch repair protein MLH3, MutL protein homolog 3, P2550, YPL164C
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High impact information on MLH3

  • Meiotic arrest and aneuploidy in MLH3-deficient mice [1].
  • Taken together, these data imply modulation of a basic Mlh1 function via combination with the three other MutL homologs and suggest specifically that Mlh1 combines with Mlh3 to promote meiotic crossing-over [2].
  • Conversely, crossing-over in the mlh3 mutant is reduced to approximately 70% of wild-type levels but correction of heteroduplex is normal [2].
  • The Saccharomyces cerevisiae MLH3 gene functions in MSH3-dependent suppression of frameshift mutations [3].
  • Combination of mutations in MLH3 and MSH6 caused a synergistic increase in the hom3-10 reversion rate, whereas the hom3-10 reversion rate in an mlh3 msh3 double mutant was the same as in the respective single mutants [3].

Biological context of MLH3


Physical interactions of MLH3


Other interactions of MLH3

  • This analysis demonstrates, for the first time, that yeast Mlh2p plays a role in the repair of mutational intermediates, and extends earlier results implicating Mlh3p in mismatch repair [5].
  • Similar results were observed when the accumulation of mutations at frameshift hot spots in the LYS2 gene was analyzed, although mutation of MLH3 did not cause the same extent of affect at every LYS2 frameshift hot spot [3].


  1. Meiotic arrest and aneuploidy in MLH3-deficient mice. Lipkin, S.M., Moens, P.B., Wang, V., Lenzi, M., Shanmugarajah, D., Gilgeous, A., Thomas, J., Cheng, J., Touchman, J.W., Green, E.D., Schwartzberg, P., Collins, F.S., Cohen, P.E. Nat. Genet. (2002) [Pubmed]
  2. Functional specificity of MutL homologs in yeast: evidence for three Mlh1-based heterocomplexes with distinct roles during meiosis in recombination and mismatch correction. Wang, T.F., Kleckner, N., Hunter, N. Proc. Natl. Acad. Sci. U.S.A. (1999) [Pubmed]
  3. The Saccharomyces cerevisiae MLH3 gene functions in MSH3-dependent suppression of frameshift mutations. Flores-Rozas, H., Kolodner, R.D. Proc. Natl. Acad. Sci. U.S.A. (1998) [Pubmed]
  4. Supercomplex formation between Mlh1-Mlh3 and Sgs1-Top3 heterocomplexes in meiotic yeast cells. Wang, T.F., Kung, W.M. Biochem. Biophys. Res. Commun. (2002) [Pubmed]
  5. Discrete in vivo roles for the MutL homologs Mlh2p and Mlh3p in the removal of frameshift intermediates in budding yeast. Harfe, B.D., Minesinger, B.K., Jinks-Robertson, S. Curr. Biol. (2000) [Pubmed]
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