Cuatrecasas P, Segal S
Science. 1966 Jul 29;153(3735):549-51. doi: 10.1126/science.153.3735.549.
Galactose dehydrogenase, a soluble enzyme of the mammalian liver, catalyzes the conversion of galactose to galactonic acid. This reaction, together with the subsequent formation of 3-ketogalactonic acid which can be decarboxylated to yield d-xylulose, is a newly discovered pathway for the metabolism of galactosee. This pathway may account for the oxidation of galactose observed in pathients with galactosemia.
半乳糖脱氢酶是哺乳动物肝脏中的一种可溶性酶,催化半乳糖转化为半乳糖酸。该反应与随后形成的可脱羧生成D-木酮糖的3-酮基半乳糖酸一起,是新发现的半乳糖代谢途径。这条途径可能解释了半乳糖血症患者体内观察到的半乳糖氧化现象。