摘要
高草酸尿症,草酸尿的排泄过多,是世界范围内的重要健康问题。草酸代谢紊乱与高草酸尿有关,因此,草酸生物合成中的酶促紊乱可导致原发性高草酸尿。丙氨酸-乙醛酸氨基转移酶-1和乙醛酸还原酶是涉及乙醛酸(草酸盐的前体)代谢的酶,与原发性高草酸尿症有关。一些研究表明,其他酶,例如乙醇酸氧化酶和丙氨酸-乙醛酸转氨酶-2,也可能与原发性高草酸尿症有关,但临床病例与基因突变之间没有明确的联系。仍然存在一些特发性高草酸尿症,需要对病因进行进一步研究。一些氨基转移酶,特别是犬尿氨酸氨基转移酶,可以将乙醛酸酯转化为甘氨酸。根据生化和结构特征,表达水平以及某些氨基转移酶的亚细胞定位,其中许多氨基酸似乎能够比丙氨酸乙醛酸氨基转移酶-1更有效地催化乙醛酸向甘氨酸的转氨作用。这项简短审查的目的是探讨原发性高草酸尿症的其他破坏性原因,并促进对导致该疾病的潜在机制的全面理解的研究。在这里,我们审查了肝中所有氨基转移酶在乙醛酸代谢中的功能。特别是,犬尿氨酸转氨酶-I和III关于其生化和结构特征,细胞定位和酶抑制作用进行了仔细讨论。迄今为止,Kynurenine氨基转移酶-III是在哺乳动物肝脏中将乙醛酸转氨为乙醛酸的最有效的线粒体酶,这可能是在原发性高草酸尿症的高草酸尿病病因中寻找的一种有趣的酶,应仔细研究其与草酸代谢的关系。 。
关键词: 高草酸尿症,草酸盐代谢,转氨酶,乙醛酸,犬尿氨酸转氨酶,肾结石。
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