Glasgow A M, Chase H P
Pediatr Res. 1976 Jul;10(7):683-6. doi: 10.1203/00006450-197607000-00010.
Propionic acid significantly inhibited 14CO2 production from [1-14C] palmitate at a concentration of 10 muM in control fibroblasts and 100 muM in methylmalonic fibroblasts. This inhibition was similar to that produced by 4-pentenoic acid. Methylmalonic acid also inhibited 14CO2 production from [1-14C] palmitate, but only at a concentration of 1 mM in control cells and 5 mM in methylmalonic cells. Propionic acid (5 mM) also inhibited ureagenesis in rat liver slices when ammonia was the substrate but not with aspartate and citrulline as substrates. Propionic acid had no direct effect on either carbamyl phosphate synthetase or ornithine transcarbamylase. These findings may explain the fatty degeneration of the liver and the hyperammonemia in propionic and methylmalonic acidemia.
在对照成纤维细胞中，丙酸在浓度为10μM时可显著抑制[1-¹⁴C]棕榈酸生成¹⁴CO₂，在甲基丙二酸血症成纤维细胞中该浓度为100μM。这种抑制作用与4-戊烯酸产生的抑制作用相似。甲基丙二酸也能抑制[1-¹⁴C]棕榈酸生成¹⁴CO₂，但仅在对照细胞中浓度为1mM时以及甲基丙二酸血症细胞中浓度为5mM时才有此作用。当以氨为底物时，丙酸（5mM）也会抑制大鼠肝切片中的尿素生成，但以天冬氨酸和瓜氨酸为底物时则不会。丙酸对氨甲酰磷酸合成酶或鸟氨酸转氨甲酰酶均无直接作用。这些发现或许可以解释丙酸血症和甲基丙二酸血症中肝脏的脂肪变性及高氨血症。
