Chibisov I V, Rozenfel'd E L, Chistova L V, Leont'ev A F
Vopr Med Khim. 1979 Jan-Feb;25(1):47-9.
Concentration of glycogen and activity of enzymes, participating in its metabolism, were studied in punctates of liver tissue obtained from three patients with clinical manifestations of hepatic forms of glycogenosis. Accumulation of glycogen in liver tissue of the patients was caused by distinct decrease in the phosphorylase activity. In two patients low activity of phosphorylase A was due to impairment of one of the enzymes, activating the phosphorylase in liver tissue, and in one patient phosphorylase B itself exhibited low activity. The in vitro data on glycogen metabolism were correlated with those, obtained in study of glycogen turnover in vivo, after loading with adrenaline. The data obtained enable to develop methods for treatment of the patients with impairments in activity of liver phosphorylase.
对三名有肝糖原累积症临床表现患者的肝组织穿刺样本中糖原浓度以及参与其代谢的酶活性进行了研究。患者肝组织中糖原的积累是由磷酸化酶活性明显降低所致。在两名患者中，磷酸化酶A活性低是由于肝脏组织中激活磷酸化酶的一种酶受损，而在一名患者中，磷酸化酶B本身活性就低。体外糖原代谢数据与肾上腺素负荷后体内糖原周转研究中获得的数据相关。所获数据有助于开发治疗肝脏磷酸化酶活性受损患者的方法。
