5-氨基酮戊酸合酶：催化血红素生物合成的第一步。
5-aminolevulinate synthase: catalysis of the first step of heme biosynthesis.
作者信息
Hunter G A, Ferreira G C
机构信息
Department of Molecular Medicine, College of Medicine, University of South Florida, Tampa, Florida 33612, USA.
出版信息
Cell Mol Biol (Noisy-le-grand). 2009 Feb 16;55(1):102-10.
Abstract
5-Aminolevulinate synthase is a homodimeric pyridoxal 5'-phosphate-dependent enzyme that catalyzes the first step of the heme biosynthetic pathway in animals, fungi, and the alpha-subclass of the photosynthetic purple bacteria. The reaction cycle involves condensation of glycine with succinyl-coenzyme A to yield 5-aminolevulinate, carbon dioxide, and CoA. Mutations in the human erythroid-specific aminolevulinate synthase gene are associated with the erythropoietic disorder X-linked sideroblastic anemia. Recent kinetic and crystallographic data have facilitated an unprecedented understanding of how this important enzyme produces 5-aminolevulinate, and suggest possible directions for future research that may lead to treatments not only for X-linked sideroblastic anemia, but also other diseases.
摘要
5-氨基乙酰丙酸合酶是一种同二聚体的依赖于磷酸吡哆醛的酶，它催化动物、真菌以及光合紫色细菌α亚类中血红素生物合成途径的第一步。该反应循环涉及甘氨酸与琥珀酰辅酶A的缩合反应，生成5-氨基乙酰丙酸、二氧化碳和辅酶A。人类红细胞特异性氨基乙酰丙酸合酶基因突变与红细胞生成障碍性X连锁铁粒幼细胞贫血相关。最近的动力学和晶体学数据极大地促进了人们对这种重要酶如何产生5-氨基乙酰丙酸的前所未有的理解，并为未来的研究指明了可能的方向，这些研究不仅可能带来针对X连锁铁粒幼细胞贫血的治疗方法，也可能用于治疗其他疾病。
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