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Mut −/−;Tg INS-CBA-G715V mouse (MUT G715V transgenic rescue)

The INS-CBA-G715V transgene on the Mut −/− background gives reduced MCM activity with moderate methylmalonic acid elevation and a normal lifespan.

Model identity

Model name
Mut −/−;Tg INS-CBA-G715V mouse (MUT G715V transgenic rescue)
Species
mouse
Genotype / zygosity
Mut −/−; Tg INS-CBA-G715V
Allele
G715V mutant Mmut transgene under insulin/CBA expression cassette

Biological characteristics

Research questions
How partial reduction of MCM activity models intermediate methylmalonic acidemia and its metabolic phenotype.
Limitations
Transgene-driven activity reduction depends on expression cassette distribution; metabolite elevation is moderate relative to mut0 mice.

References

  1. Genetically engineered animal models of methylmalonic acidemia review (PMID:41239408)
  2. ARRIVE 2.0 guidelines