Maddaiah V T
Biochem Biophys Res Commun. 1985 Mar 15;127(2):565-70. doi: 10.1016/s0006-291x(85)80197-2.
NH4C1 inhibited oxygen consumption (State 3, ADP induced) by rat liver mitochondria respiring on palmitoyl-L-carnitine or octanoic acid but not on succinate or malate + glutamate. The inhibition became apparent at 0.02 mM reaching a plateau (40%) at 2 mM NH4C1. Similar inhibition was observed with uncoupled (in the presence of 2, 4-dinitrophenol) mitochondria. The inhibition of uncoupled mitochondria was reversible as the rate of respiration with palmitoyl-L-carnitine was further increased by succinate and the total rate was unaffected by NH4C1. Therefore, NH+4 inhibition of mitochondrial respiration may lead to fatty infiltration and be one of the causes of the pathophysiology in children with Reye's syndrome and disorders of urea cycle enzymes.
氯化铵抑制大鼠肝脏线粒体在以棕榈酰-L-肉碱或辛酸为呼吸底物时的氧消耗（状态3，由ADP诱导），但对以琥珀酸或苹果酸+谷氨酸为呼吸底物时无抑制作用。在0.02 mM时抑制作用开始明显，在2 mM氯化铵时达到平台期（40%）。在解偶联（存在2,4-二硝基苯酚）的线粒体中也观察到类似的抑制作用。解偶联线粒体的抑制作用是可逆的，因为琥珀酸可进一步提高以棕榈酰-L-肉碱为底物时的呼吸速率，且总速率不受氯化铵影响。因此，铵离子对线粒体呼吸的抑制可能导致脂肪浸润，是瑞氏综合征患儿及尿素循环酶紊乱病理生理机制的原因之一。
