丙酸代谢
中文名称
通路描述
丙酸代谢将丙酰辅酶A转化为甲基丙二酰辅酶A，然后转化为琥珀酰辅酶A以进入三羧酸循环，该过程需要维生素B12作为辅因子；缺陷会导致有机酸血症，伴有严重的神经系统症状。
英文描述
Propanoate metabolism converts propionyl-CoA into methylmalonyl-CoA then to succinyl-CoA for TCA entry via methylmalonyl-CoA mutase requiring vitamin B12 as cofactor; defects cause organic acidemias with severe neurological symptoms.
所含基因
32 个基因