苹果酸代谢和甘氨酸降解
中文名称
通路描述
苹果酸是在甘氨酸和羟脯氨酸降解过程中产生的，可以转化为草酸。在人类中，该过程发生在肝脏。两个苹果酸代谢酶的缺陷（丙氨酸：苹果酸氨基转移酶 AGXT 和甘油酸脱氢酶/苹果酸还原酶 GRHPR）与过量的草酸产生相关（Danpure 2005）。人类中甘氨酸、乳酸和苹果酸之间的反应以及将苹果酸转化为草酸的反应在分子水平上已得到详细表征。从部分纯化的大鼠和牛肝脏提取物中推断出从羟脯氨酸到苹果酸的反应序列，但参与相应人类反应的酶尚未鉴定。
英文描述
Glyoxylate metabolism and glycine degradation Glyoxylate is generated in the course of glycine and hydroxyproline catabolism and can be converted to oxalate. In humans, this process takes place in the liver. Defects in two enzymes of glyoxylate metabolism, alanine:glyoxylate aminotransferase (AGXT) and glycerate dehydrogenase/glyoxylate reductase (GRHPR), are associated with pathogenic overproduction of oxalate (Danpure 2005). The reactions that interconvert glycine, glycolate, and glyoxylate and convert glyoxylate to oxalate have been characterized in molecular detail in humans. A reaction sequence for the conversion of hydroxyproline to glyoxylate has been inferred from studies of partially purified extracts of rat and bovine liver but the enzymes involved in the corresponding human reactions have not been identified.
所含基因
11 个基因