Bird M I, Munday L A, Saggerson E D, Clark J B
Biochem J. 1985 Feb 15;226(1):323-30. doi: 10.1042/bj2260323.
Carnitine palmitoyltransferase and carnitine octanoyltransferase activities in brain mitochondrial fractions were approx. 3-4-fold lower than activities in liver. Estimated Km values of CPT1 and CPT2 (the overt and latent forms respectively of carnitine palmitoyltransferase) for L-carnitine were 80 microM and 326 microM, respectively, and K0.5 values for palmitoyl-CoA were 18.5 microM and 12 microM respectively. CPT1 activity was strongly inhibited by malonyl-CoA, with I50 values (concn. giving 50% of maximum inhibition) of approx. 1.5 microM. In the absence of other ligands, [2-14C]malonyl-CoA bound to intact brain mitochondria in a manner consistent with the presence of two independent classes of binding sites. Estimated values for KD(1), KD(2), N1 and N2 were 18 nM, 27 microM, 1.3 pmol/mg of protein and 168 pmol/mg of protein respectively. Neither CPT1 activity, nor its sensitivity towards malonyl-CoA, was affected by 72 h starvation. Rates of oxidation of palmitoyl-CoA (in the presence of L-carnitine) or of palmitoylcarnitine by non-synaptic mitochondria were extremely low, indicating that neither CPT1 nor CPT2 was likely to be rate-limiting for beta-oxidation in brain. CPT1 activity relative to mitochondrial protein increased slightly from birth to weaning (20 days) and thereafter decreased by approx. 50%.
脑线粒体组分中的肉碱棕榈酰转移酶和肉碱辛酰转移酶活性约比肝脏中的活性低3 - 4倍。肉碱棕榈酰转移酶1(CPT1)和肉碱棕榈酰转移酶2(CPT2,分别为肉碱棕榈酰转移酶的显性和隐性形式)对L - 肉碱的估计Km值分别为80微摩尔/升和326微摩尔/升,对棕榈酰辅酶A的K0.5值分别为18.5微摩尔/升和12微摩尔/升。CPT1活性受到丙二酰辅酶A的强烈抑制,I50值(产生50%最大抑制的浓度)约为1.5微摩尔/升。在没有其他配体的情况下,[2 - 14C]丙二酰辅酶A以与存在两类独立结合位点一致的方式结合到完整的脑线粒体上。KD(1)、KD(2)、N1和N2的估计值分别为18纳摩尔/升、27微摩尔/升、1.3皮摩尔/毫克蛋白质和168皮摩尔/毫克蛋白质。72小时饥饿既不影响CPT1活性,也不影响其对丙二酰辅酶A的敏感性。非突触线粒体对棕榈酰辅酶A(在L - 肉碱存在下)或棕榈酰肉碱的氧化速率极低,这表明CPT1和CPT2都不太可能是脑中β - 氧化的限速因素。相对于线粒体蛋白的CPT1活性从出生到断奶(20天)略有增加,此后下降约50%。