支链氨基酸的全身代谢命运。
Whole-body metabolic fate of branched-chain amino acids.
作者信息
Blair Megan C, Neinast Michael D, Arany Zoltan
机构信息
Cardiovascular Institute, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, U.S.A.
Lewis-Sigler Institute for Integrative Genomics, Princeton University, Princeton, NJ, U.S.A.
出版信息
Biochem J. 2021 Feb 26;478(4):765-776. doi: 10.1042/BCJ20200686.
Abstract
Oxidation of branched-chain amino acids (BCAAs) is tightly regulated in mammals. We review here the distribution and regulation of whole-body BCAA oxidation. Phosphorylation and dephosphorylation of the rate-limiting enzyme, branched-chain α-ketoacid dehydrogenase complex directly regulates BCAA oxidation, and various other indirect mechanisms of regulation also exist. Most tissues throughout the body are capable of BCAA oxidation, and the flux of oxidative BCAA disposal in each tissue is influenced by three key factors: 1. tissue-specific preference for BCAA oxidation relative to other fuels, 2. the overall oxidative activity of mitochondria within a tissue, and 3. total tissue mass. Perturbations in BCAA oxidation have been implicated in many disease contexts, underscoring the importance of BCAA homeostasis in overall health.
摘要
在哺乳动物中，支链氨基酸（BCAAs）的氧化受到严格调控。我们在此综述全身BCAA氧化的分布与调控。限速酶支链α-酮酸脱氢酶复合体的磷酸化和去磷酸化直接调节BCAA氧化，并且还存在各种其他间接调控机制。全身大多数组织都能够进行BCAA氧化，每个组织中BCAA氧化处置的通量受三个关键因素影响：1. 相对于其他燃料，组织对BCAA氧化的特异性偏好；2. 组织中线粒体的整体氧化活性；3. 组织总质量。BCAA氧化的紊乱与许多疾病情况有关，这突出了BCAA内稳态在整体健康中的重要性。
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