Carnitine palmitoyltransferase I - Wikipedia
Carnitine palmitoyltransferase I (CPT1) also known as carnitine acyltransferase I, CPTI, CAT1, CoA:carnitine acyl transferase (CCAT), or palmitoylCoA transferase I, is a mitochondrial enzyme responsible for the formation of acyl carnitines by catalyzing the transfer of the acyl group of a long-chain fatty acyl-CoA from coenzyme A to l-carnitine.
线粒体长链脂肪酸氧化障碍：肉碱棕榈酰转移酶 I 缺乏症（CPT1A
2023年11月14日 · 所谓的肉碱穿梭介导长链脂肪酰基物质从细胞质运输到线粒体中，通过β-氧化产生能量。 线粒体外膜上的肉碱棕榈酰转移酶 I (CPT I) 将长链酰基辅酶 A 转化为其酰基肉碱等价物，这些等价物通过肉碱-酰基肉碱转位酶转运到线粒体内室，然后由 CPT II 重新转化为酰基辅酶 A线粒体内膜 ，因此，CPT I 是长链脂肪酸进入线粒体进行 β-氧化的限速因子。 在CPT1A双等位基因致病变异引起的CPT I活性降低中，脂肪酸无法进入线粒体进行能量产生；结果是出现禁食 …
Carnitine Palmitoyltransferase Deficiency - Cedars-Sinai
What is carnitine palmitoyltransferase deficiency? Carnitine palmitoyltransferase (CPT) deficiency is a very rare condition that causes muscle weakness and other symptoms. It happens because of a problem with one of two enzymes, CPT1 or CPT2. Enzymes are substances in the body that help cause chemical reactions.
Carnitine palmitoyltransferase I deficiency - MedlinePlus
Carnitine palmitoyltransferase I (CPT I) deficiency is a condition that prevents the body from using certain fats for energy, particularly during periods without food (fasting). Explore symptoms, inheritance, genetics of this condition.
Fatty acid oxidation and carnitine palmitoyltransferase I: …
Among these targets, the carnitine palmitoyltransferase system is responsible for delivering the long-chain fatty acid (FA) from cytoplasm into mitochondria for oxidation, where carnitine palmitoyltransferase I (CPTI) catalyzes the rate-limiting step of fatty acid oxidation (FAO).
Carnitine Palmitoyltransferase 1A Deficiency - GeneReviews®
2005年7月27日 · Carnitine palmitoyltransferase 1A (CPT1A) deficiency is a disorder of long-chain fatty acid oxidation. Clinical manifestations usually occur in an individual with a concurrent febrile or gastrointestinal illness when energy demands are …
Carnitine Palmitoyltransferase II Deficiency - GeneReviews®
2004年8月27日 · Carnitine palmitoyltransferase II (CPT II) deficiency is a disorder of long-chain fatty-acid oxidation. The three clinical presentations are lethal neonatal form, severe infantile hepatocardiomuscular form, and myopathic form (which is usually mild and can manifest from infancy to adulthood).
Carnitine Palmitoyltransferase I - an overview - ScienceDirect
Carnitine Palmitoyltransferase I is a group of enzymes with tissue-specific isoforms, including liver (CPT-IA), muscle (CPT-IB), and brain (CPT-IC) isoforms. Deficiency of the liver enzyme can lead to severe hepatic encephalopathy. AI generated definition based on: Neurology and Clinical Neuroscience, 2007
Carnitine palmitoyltransferase 1C regulates cancer cell …
2018年1月9日 · Here we show that carnitine palmitoyltransferase 1C (CPT1C), an enzyme that catalyzes carnitinylation of fatty acids for transport into mitochondria for β-oxidation, plays a major role in the...
Carnitine Palmitoyltransferase System: A New Target for Anti ...
2021年10月26日 · As one of the most important lipid metabolic pathways, fatty acid oxidation (FAO) and its key rate-limiting enzyme, the carnitine palmitoyltransferase (CPT) system, regulate host immune responses and thus are of great clinical significance.