Abstract: Pantothenic acid，also known as vitamin B5，is an essential vitamin for the biosynthesis of coenzyme A（CoA）and acyl carrier protein（ACP）in mammalian cells. It participates in the synthesis or catabolism of fatty acids，carbohydrates，cholesterol，acetylcholine and other metabolites through the synthesis of CoA and ACP. The absorption of pantothenic acid is dependent on a membrane transport system that is shared with other compounds，including biotins. Impairment of this transport system or its metabolic processes can result in mitochondrial dysfunction and metabolic disorders. At present，there is no established recommended intake volume for pantothenic acid. Although primary pantothenic acid deficiency is uncommon，  secondary pantothenic acid deficiency is common，such as in the patients with PANK2 gene mutations and mitochondrial diseases. Pantothenic acid deficiency can result in multi-system damage，and the clinical presentation is not specific. The pantothenic acid supplem-entation is helpful for the prevention and treatment of many rare and common diseases.

Key words: pantothenic acid, vitamin B5, pantothenic acid deficiency, pantothenate kinase, mitochondria

摘要： 泛酸又称维生素B5，是哺乳动物细胞中辅酶A（coenzyme A，CoA）和酰基载体蛋白（acyl carrier protein，ACP）生物合成所必需的维生素，通过合成CoA和ACP，参与脂肪酸、碳水化合物、胆固醇、乙酰胆碱等合成或分解代谢。泛酸的吸收与生物素等共用一种膜转运系统，其转运或代谢障碍可导致线粒体功能损害及代谢紊乱。目前，对于泛酸尚无标准化的推荐摄入量，虽然原发性泛酸缺乏症罕见，但是继发性泛酸缺乏较为常见，如PANK2基因突变及线粒体病患者常存在泛酸缺乏。泛酸缺乏可导致多系统损害，且临床表现无特异性。补充泛酸有助于防治多种罕见及常见疾病。

关键词: 泛酸, 维生素B5, 泛酸缺乏症, 泛酸激酶, 线粒体