Lemonnier A, Moatti N, Baussan C
C R Seances Soc Biol Fil. 1979;173(2):483-95.
Glycogen synthesis and breakdown in the liver are tightly controlled through different mechanisms. The purpose of this review is to describe some properties of the enzymes involved in the glycogen metabolism and the sequence of events by which glucose, allosteric effectors and hormones control this metabolism in the liver. Clinical, genetic and biological aspects of the phosphorylase and the phosphorylase kinase deficiencies are examined. The enzymatic analysis of the haemolysates from the patients allows discrimination of these two types of glycogenosis.
肝脏中糖原的合成与分解通过不同机制受到严格调控。本综述的目的是描述参与糖原代谢的酶的一些特性，以及葡萄糖、别构效应剂和激素在肝脏中控制这种代谢的一系列事件。对磷酸化酶和磷酸化酶激酶缺乏症的临床、遗传和生物学方面进行了研究。对患者溶血产物进行酶分析可区分这两种糖原贮积病类型。
