从维生素B12的氰callamin形式中去除氰化物的取决于贩运伴侣，该伴侣可能不存在某些个体，谁会对含水蛋白蛋白酶的反应更好

60年前发现的氰化物组B（12）或氰基bobalamin如何在60年前发现的氰化物组的奥秘被驱逐出来。 反应。从NADPH通过胞质黄蛋白氧化还原酶从NADPH转移的电子用于裂解钴 - 碳键，并还原消除氰化物配体。该产物COB（II）alamin是一种已知的底物，用于吸收活性辅因子形式，甲基cobalamin和5-脱氧腺苷cobalamin，AD与两个B（12）依赖性靶酶，蛋氨酸合酶和甲基氨基甲基-COA突变酶所需的“碱基”状态结合。 Defects in MMACHC represent the most common cause of inborn errors of B(12) metabolism, and our results explain the observation that fibroblasts from these patients are poorly responsive to vitamin B(12) but show some metabolic correction with aquocobalamin, a cofactor form lacking the cyanide ligand, which is mirrored by patients showing poorer clinical responsiveness to cyano- versus Aquocobalamin。