Mut −/− mouse (mut0 methylmalonic acidemia)
Mut −/− mice lack methylmalonyl-CoA mutase (MCM) activity, accumulate methylmalonic acid, methylcitric acid and C3 acylcarnitine, and die within 24 hours of birth, reproducing the mut0 form of methylmalonic acidemia.
模型身份
- 模型名称
- Mut −/− mouse (mut0 methylmalonic acidemia)
- 物种
- mouse
- 基因型／合子状态
- Mut −/−
- 等位基因
- exon 3 of Mmut replaced by antibiotic selection marker
生物学特性
- 适用研究问题
- What genetically engineered animal models exist for methylmalonic acidemia and what are their characteristics.
- 模型局限
- Neonatal lethality restricts study to the immediate postnatal window or to intervention experiments begun before birth.