酪氨酸分解代谢
中文名称
通路描述
细胞质中的酪氨酸经转氨基作用形成3-(4-羟基苯基)丙酮酸,随后经过四步反应转化为草酸和乙酰乙酸。因此,酪氨酸既是糖原代谢的前体(草酸),又是酮体代谢的前体(乙酰乙酸)。任何步骤的缺陷均会导致代谢疾病,表现为酪氨酸积累(酪氨酸血症)(Mitchell et al. 2001; Held, 2006)。
英文描述
Tyrosine catabolism Cytosolic tyrosine is transaminated to form 3-(4-hydroxyphenyl)pyruvate which in four further reactions is converted to fumarate and acetoacetate. Tyrosine is thus both a glucogenic (fumarate) and a ketogenic (acetoacetate) amino acid. Defects in any of the steps lead to metabolic diseases with accumulation of tyrosine (tyrosinemia) (Mitchell et al. 2001; Held, 2006).
所含基因
5 个基因