过去认为天然生物素缺乏在人类极为罕见,近来发现,生物素或生物素依赖的结合羧化酶缺乏病逐有增加,属于遗传性代谢病。自然界存在有α-生物素(存于蛋黄中)和β-生物素(存于肝脏中),生物素与酶结合参与体内CO_2的固定和羧化过程,是许多酶(如乙酰辅酶A羧化酶、丙酰辅酶A、3-甲基丁烯酰酶A和丙酮酸的羧化酶)必须的一种羧化酶辅酶的成 In the past that the lack of natural biotin in humans is extremely rare, recently found that biotin or biotin dependent combination of carboxylase deficiency by an increase, are inherited metabolic disease. Biotin (stored in the egg yolk) and β-biotin (stored in the liver) exist in nature. The binding of biotin and enzymes is involved in the fixation and carboxylation of CO 2 in the body. Many enzymes, such as acetyl-CoA carboxylation Enzymes, propionyl-CoA, 3-methylcrotonoylase A and pyruvate carboxylase)