2-羟基谷氨酸与2-羟基戊二酸之间的相互转化
中文名称
通路描述
2-羟基戊二酸的两对立体异构体通常在线粒体基质中转化为2-羟基戊二酸，然后进入三羧酸循环代谢。2-羟基戊二酸的生理来源尚未确定，但合理的假设是它由赖氨酸分解产生或作为δ-氨基levulinate代谢的副产物。立体异构体在FAD依赖的反应中被氧化为2-羟基戊二酸，由D2HGDH（特异性识别R(-)-2-羟基戊二酸）和L2HGDH（特异性识别S(-)-2-羟基戊二酸）催化。任一酶的遗传缺陷与2-羟基戊二酸的积累和不同程度的神经系统症状相关。R(-)-2-羟基戊二酸可逆地与琥珀酸半醛反应形成4-羟基丁酸和2-羟基戊二酸，由ADHFE1催化。尚未发现该酶的缺陷患者患有2-羟基戊二酸水平升高（Struys 2006）。
英文描述
Interconversion of 2-oxoglutarate and 2-hydroxyglutarate The two stereoisomers of 2-hydroxyglutarate are normally converted to 2-oxoglutarate in the mitochondrial matrix, and can then be metabolized by the citric acid cycle. The physiological sources of 2-hydroxyglutarate have not been established although plausible hypotheses are that it is generated by lysine breakdown or as a byproduct of delta-aminolevulinate metabolism. The stereoisomers are oxidized to 2-oxoglutarate in FAD-dependent reactions catalyzed by the enzymes D2HGDH (specific for R(-)-2-hydroxyglutarate) and L2HGDH (specific for S(-)-2-hydroxyglutarate). An inherited deficiency in either enzyme is associated with accumulation of 2-hydroxyglutarate and variable neurological symptoms. R(-)-2-hydroxyglutarate also reacts reversibly with succinate semialdehyde to form 4-hydroxybutyrate and 2-oxoglutarate, catalyzed by ADHFE1. No deficiencies of this enzyme have been found in patients with elevated 2-hydroxyglutarate levels (Struys 2006).
所含基因
3 个基因