Poloni M, Patrini C, Rocchelli B, Rindi G
Arch Neurol. 1982 Aug;39(8):507-9. doi: 10.1001/archneur.1982.00510200049009.
Free thiamin and thiamin monophosphate levels were determined by an electrophoretic fluorometric micromethod in plasma and CSF of patients with amyotrophic lateral sclerosis (ALS), alcoholics, and controls. In plasma of patients with ALS as well as in plasma and CSF of alcholics, both thiamin and thiamin monophosphate concentrations were decreased so that the thiamin-thiamin monophosphate (T/TMP) ratio remained unchanged compared with that of controls. In CSF of patients with ALS, however, thiamin monophosphate values decreased much more than thiamin levels, so that the T/TMP ratio was significantly increased. The selective impairment of thiamin monophosphate production by nerve cells is likely to result from the reduction of the activity of thiamin pyrophosphatase, an enzyme synthetized and highly concentratd in the Golgi complex. Thiamin pyrophosphatase is known to diminish in ALS as well as in experimental motor neuronal degeneration or axotomy. Thus, the T/TMP ratio could be taken as an index of the impairment of neuronal protein synthesis in ALS.
采用电泳荧光微量法测定了肌萎缩侧索硬化症（ALS）患者、酗酒者及对照组血浆和脑脊液中游离硫胺素和硫胺素单磷酸的水平。在ALS患者的血浆以及酗酒者的血浆和脑脊液中，硫胺素和硫胺素单磷酸的浓度均降低，因此与对照组相比，硫胺素 - 硫胺素单磷酸（T/TMP）比值保持不变。然而，在ALS患者的脑脊液中，硫胺素单磷酸值的下降幅度远大于硫胺素水平，因此T/TMP比值显著升高。神经细胞硫胺素单磷酸生成的选择性损害可能是由于硫胺素焦磷酸酶活性降低所致，该酶在高尔基体中合成并高度浓缩。已知硫胺素焦磷酸酶在ALS以及实验性运动神经元变性或轴突切断时会减少。因此，T/TMP比值可作为ALS中神经元蛋白质合成受损的指标。
