著者

真鍋 恵子 真鍋 満久 須藤 加代子 菅野 剛史

出版者

日本電気泳動学会

雑誌

生物物理化学 (ISSN:00319082)

巻号頁・発行日

vol.26, no.6, pp.457-462, 1982-12-25

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The physico-chemical and kinetical properties of abnormal pseudocholinesterase (p-ChE) in two families (J. T. and K. K.) were studied and discussed. The p-ChE levels of the patient J. T. an d K. K. were 10% and 3% of the lower limit of normal ranges, respectively. The p-ChE of patient J.T . was classified as homozygote for atypical gene by the inhibition studies. However, the p-ChE of K. K. showed much larger Km values for several substrate s than normal controls and the Hill's number of the patient's p-ChE was 1. 10, though the v alues less than 1.0 were usually obtained from normal control. Therefore, the p-ChE of K. K. have not the allosteric property with negative cooperativity, as was the nature of normal one. Further studies by isoelectric focusing and slab PAG electrophoresis revealed the traces of the activity of p-ChE in the patient J.T ., but any activity band was not detected in the patient K. K. These results indicated that the patient J. T. was homozygote of atypical gene and that the patient K. K. was hom ozygote for silent gene. It was suggested that the analysis of Hill's number, additional to the c onventional inhibition studies, might be useful for the detection of the silent gene of p-ChE in deta ils.