关键词: 泛酸, 维生素B5, 泛酸缺乏症, 泛酸激酶, 线粒体

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