Characterization of phosphofructokinase-deficient canine erythrocytes.
Characterization of phosphofructokinase-deficient canine erythrocytes.
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磷酸果糖激酶缺陷犬红细胞的表征。
DOI：
10.1007/bf01800017
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发表时间：
1992
影响因子：
4.2
通讯作者：
Dunaway,GA
中科院分区：
文献类型：
--
作者：
Harvey,JW;Pate,MG;Mhaskar,Y;Dunaway,GA
同种型肌肉型磷酸果糖激酶（PFK）缺乏症犬的正常红细胞PFK活性约为20%，并表现出代偿性溶血性贫血。受影响犬的红细胞葡萄糖-6-磷酸和果糖-6-磷酸浓度升高，磷酸二羟丙酮和2，3-二磷酸甘油酸值低于正常值。PFK步骤远端的其他中间体未显著低于正常值，果糖-1，6-二磷酸甚至高于正常值。由于存在网织红细胞，受影响犬的红细胞ATP高于正常值。腺苷酸代谢异常表现为低ATP/AMP和ADP/AMP比值，以及受影响的红细胞与氰化物孵育时无法维持ATP含量。葡萄糖-1，6-二磷酸含量正常，受累犬红细胞中果糖-2，6-二磷酸含量高于正常。红细胞PFK同工酶的研究显示，受影响犬的酶动力学特性发生改变，这似乎是由于M型亚基丢失所致。
Dogs homozygously affected with muscle‐type phosphofructokinase (PFK) deficiency had about 20% of normal erythrocyte PFK activity and exhibited a compensated haemolytic anaemia. Erythrocyte glucose‐6‐phosphate and fructose‐6‐phosphate concentrations were increased and dihydroxyacetone phosphate and 2,3‐bisphosphoglycerate values were below normal in affected dogs. Other intermediates distal to the PFK step were not significantly below normal and fructose‐1,6‐bisphosphate was even above normal. Erythrocyte ATP was higher than normal in affected dogs owing to the reticulocytes present. Abnormal adenylate metabolism was demonstrated by low ATP/AMP and ADP/AMP ratios and the inability to maintain ATP content when affected erythrocytes were incubated with cyanide. Glucose‐1,6‐bisphosphate content was normal, and fructose‐2,6‐bisphosphate content in affected canine erythrocytes was higher than normal. Studies of erythrocyte PFK isozymes revealed altered enzyme kinetic properties in affected dogs which appeared to be due to the loss of the M‐type subunit.