scholarly journals Wheat-bran autolytic peptides containing a branched-chain amino acid attenuate non-alcoholic steatohepatitis via the suppression of oxidative stress and the upregulation of AMPK/ACC in high-fat diet-fed mice

2016 ◽  
Vol 39 (2) ◽  
pp. 407-414 ◽  
Author(s):  
Takumi Kawaguchi ◽  
Takato Ueno ◽  
Yoichi Nogata ◽  
Masako Hayakawa ◽  
Hironori Koga ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Amino Acid ◽  
Wheat Bran ◽  
High Fat Diet ◽  
High Fat ◽  
Alcoholic Steatohepatitis ◽  
Branched Chain Amino Acid ◽  
Branched Chain

scholarly journals Altered Redox Homeostasis in Branched-Chain Amino Acid Disorders, Organic Acidurias, and Homocystinuria

2018 ◽  
Vol 2018 ◽  
pp. 1-17 ◽  
Author(s):  
Eva Richard ◽  
Lorena Gallego-Villar ◽  
Ana Rivera-Barahona ◽  
Alfonso Oyarzábal ◽  
Belén Pérez ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Amino Acid ◽  
Mitochondrial Dysfunction ◽  
Redox Signaling ◽  
Acid Oxidation ◽  
Published Data ◽  
Mitochondrial Enzymes ◽  
Organic Acidurias ◽  
Branched Chain Amino Acid ◽  
Branched Chain

Inborn errors of metabolism (IEMs) are a group of monogenic disorders characterized by dysregulation of the metabolic networks that underlie development and homeostasis. Emerging evidence points to oxidative stress and mitochondrial dysfunction as major contributors to the multiorgan alterations observed in several IEMs. The accumulation of toxic metabolites in organic acidurias, respiratory chain, and fatty acid oxidation disorders inhibits mitochondrial enzymes and processes resulting in elevated levels of reactive oxygen species (ROS). In other IEMs, as in homocystinuria, different sources of ROS have been proposed. In patients’ samples, as well as in cellular and animal models, several studies have identified significant increases in ROS levels along with decreases in antioxidant defences, correlating with oxidative damage to proteins, lipids, and DNA. Elevated ROS disturb redox-signaling pathways regulating biological processes such as cell growth, differentiation, or cell death; however, there are few studies investigating these processes in IEMs. In this review, we describe the published data on mitochondrial dysfunction, oxidative stress, and impaired redox signaling in branched-chain amino acid disorders, other organic acidurias, and homocystinuria, along with recent studies exploring the efficiency of antioxidants and mitochondria-targeted therapies as therapeutic compounds in these diseases.

scholarly journals Branched-Chain Amino Acid Supplementation Reduces Oxidative Stress and Prolongs Survival in Rats with Advanced Liver Cirrhosis

PLoS ONE ◽  
2013 ◽  
Vol 8 (7) ◽  
pp. e70309 ◽  
Author(s):  
Motoh Iwasa ◽  
Yoshinao Kobayashi ◽  
Rumi Mifuji-Moroka ◽  
Nagisa Hara ◽  
Hirohide Miyachi ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Liver Cirrhosis ◽  
Amino Acid ◽  
Amino Acid Supplementation ◽  
Acid Supplementation ◽  
Branched Chain Amino Acid ◽  
Advanced Liver Cirrhosis ◽  
Branched Chain
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