Pyruvate dehydrogenase deficiency (also known as pyruvate dehydrogenase complex deficiency or PDCD or PDH deficiency) is a rare neurodegenerative disorder associated with abnormal …

注意缺陷与多动障碍（ADHD）是一种以注意力无法持久集中、过度活跃和情绪易冲动为主症的神经发育障碍。 常在儿童时期发病，多数在学龄前期开始出现，主要分为儿童青少年ADHD和成 …

Pyruvate dehydrogenase deficiency is characterized by the buildup of a chemical called lactic acid in the body and a variety of neurological problems. Signs and symptoms of this condition …

Pyruvate dehydrogenase complex (PDC) deficiency is a type of metabolic disease. This means that the body is not able to efficiently break down nutrients in food to be used for energy. …

2021年6月17日 · Primary pyruvate dehydrogenase complex deficiency (PDCD) is a mitochondrial disorder of carbohydrate oxidation that mostly affects the brain and leads to decreased ATP …

PDH是典型的多酶体系，将催化连续反应的几种酶结合成复杂有序的结构，以提高效率。 原核生物的复合体较为简单，大肠杆菌的PDH含有12个E1二聚体，24个E2和6个E3二聚体。 真核生物 …

2020年1月17日 · 丙酮酸脱氢酶缺乏症（PDHD）是一种线粒体能量代谢异常的遗传性疾病，由基因突变引起丙酮酸脱氢酶活性降低的一组疾病，多在婴幼儿期起病。 自1970年首例先天性报 …

2021年6月14日 · Learn about Pyruvate Dehydrogenase Complex Deficiency, including symptoms, causes, and treatments. If you or a loved one is affected by this condition, visit.

Pyruvate dehydrogenase deficiency (PDHD) is a rare neurometabolic disorder characterized by a wide range of clinical signs with metabolic and neurological

丙酮酸脱氢酶复合物 (PDH)将柠檬酸循环和随后的氧化磷酸化与糖酵解、糖异生以及脂质和氨基酸代谢途径联系起来。 PDH催化丙酮酸的氧化脱羧生成乙酰辅酶A (乙酰辅酶A)、NADH和CO2 …