The Mlh3, mutL homolog 3 (E coli), gene encodes a DNA mismatch repair protein involved in mammalian meiotic recombination. These mice carry a targeted mutation of Mlh3 in which exon 2 has been replaced by sequence encoding lacZ and a floxed PGK-NEO cassette. Mice homozygous for this targeted mutation are viable but sterile, and exhibit increased microsatellite instability rate. Heterozygotes are viable and fertile.
Spermatocytes of homozygous males arrest at pachytene, diplonema and metaphase stages, resulting in azoospermia. Homozygous males exhibit small testis size when compared to wildtype controls. Oocytes of homozygous females fail to complete meiosis after fertilization. Gametes from homozygotes exhibit microsatellite instability and aneuploidy, in addition to meiotic arrest. No gene product (mRNA) is detected by Northern blot analysis of testes from homozygotes. 
Homozygotes on the 129 Sv/Ev have a shortened life span, with a mean life span of 15.1 months.
An increased frequency of tumors of the gastrointestinal tract and other tissues is observed in homozygotes: more than 50% of homozygotes developed gastrointestinal tumors.

In this strain exon 2 of the Mlh3, mutL homolog 3 (E coli), gene is disrupted. Homozygotes are infertile, exhibiting meiotic arrest during gametogenesis, and develop a higher frequency of gastrointestinal tumors when compared to controls. This mutant mouse strain may be useful in studies of microsatellite instability, DNA-damage response, and tumorigenesis.

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