Morris Claudia R, Hamilton-Reeves Jill, Martindale Robert G, Sarav Menaka, Ochoa Gautier Juan B
1 Department of Pediatrics, Division of Pediatric Emergency Medicine, Emory-Children's Center for Cystic Fibrosis and Airways Disease Research, Emory University School of Medicine, Atlanta, Georgia, USA.
2 Department of Dietetics and Nutrition, University of Kansas, Kansas City, Kansas, USA.
Nutr Clin Pract. 2017 Apr;32(1_suppl):30S-47S. doi: 10.1177/0884533617691250. Epub 2017 Feb 1.
Nonessential amino acids are synthesized de novo and therefore not diet dependent. In contrast, essential amino acids must be obtained through nutrition since they cannot be synthesized internally. Several nonessential amino acids may become essential under conditions of stress and catabolic states when the capacity of endogenous amino acid synthesis is exceeded. Arginine and glutamine are 2 such conditionally essential amino acids and are the focus of this review. Low arginine bioavailability plays a pivotal role in the pathogenesis of a growing number of varied diseases, including sickle cell disease, thalassemia, malaria, acute asthma, cystic fibrosis, pulmonary hypertension, cardiovascular disease, certain cancers, and trauma, among others. Catabolism of arginine by arginase enzymes is the most common cause of an acquired arginine deficiency syndrome, frequently contributing to endothelial dysfunction and/or T-cell dysfunction, depending on the clinical scenario and disease state. Glutamine, an arginine precursor, is one of the most abundant amino acids in the body and, like arginine, becomes deficient in several conditions of stress, including critical illness, trauma, infection, cancer, and gastrointestinal disorders. At-risk populations are discussed together with therapeutic options that target these specific acquired amino acid deficiencies.
非必需氨基酸可从头合成,因此不依赖饮食。相比之下,必需氨基酸必须通过营养获取,因为它们无法在体内合成。在应激和分解代谢状态下,当内源性氨基酸合成能力被超过时,几种非必需氨基酸可能会变成必需氨基酸。精氨酸和谷氨酰胺就是这样两种条件必需氨基酸,也是本综述的重点。低精氨酸生物利用度在越来越多的各种疾病的发病机制中起关键作用,包括镰状细胞病、地中海贫血、疟疾、急性哮喘、囊性纤维化、肺动脉高压、心血管疾病、某些癌症和创伤等。精氨酸酶对精氨酸的分解代谢是获得性精氨酸缺乏综合征最常见的原因,根据临床情况和疾病状态,常导致内皮功能障碍和/或T细胞功能障碍。谷氨酰胺是精氨酸的前体,是体内最丰富的氨基酸之一,与精氨酸一样,在几种应激状态下会缺乏,包括危重病、创伤、感染、癌症和胃肠道疾病。本文将讨论高危人群以及针对这些特定获得性氨基酸缺乏的治疗选择。