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Decreased BCAA levels in liver cirrhosis are due to increased use of the BCAA as a donor of amino group to \u03b1-ketoglutarate for synthesis of glutamate, which in muscles acts as a substrate for ammonia detoxification to glutamine. Increased BCAA levels in diabetes are due to alterations in glycolysis, citric acid cycle, and fatty acid oxidation. Decreased glycolysis and citric cycle activity impair BCAA transamination to branched-chain keto acids (BCKAs) due to decreased supply of amino group acceptors (\u03b1-ketoglutarate, pyruvate, and oxaloacetate); increased fatty acid oxidation inhibits flux of BCKA through BCKA dehydrogenase due to increased supply of NADH and acyl-CoAs. Alterations in BCAA levels in disorders with SIRS are inconsistent due to contradictory effects of SIRS on muscles. Specifically, increased proteolysis and insulin resistance tend to increase BCAA levels, whereas activation of BCKA dehydrogenase and glutamine synthesis tend to decrease BCAA levels. The studies are needed to elucidate the role of alterations in BCAA metabolism and the effects of BCAA supplementation on the outcomes of specific diseases.<\/jats:p>","DOI":"10.33549\/physiolres.934648","type":"journal-article","created":{"date-parts":[[2021,7,28]],"date-time":"2021-07-28T12:10:39Z","timestamp":1627474239000},"page":"293-305","source":"Crossref","is-referenced-by-count":58,"title":["The Role of Skeletal Muscle in The Pathogenesis of Altered Concentrations of Branched-Chain Amino Acids (Valine, Leucine, and Isoleucine) in Liver Cirrhosis, Diabetes, and Other Diseases"],"prefix":"10.33549","author":[{"given":"M","family":"Hole\u010dek","sequence":"first","affiliation":[{"name":"Department of Physiology, Faculty of Medicine in Hradec Kr\u00e1lov\u00e9, Charles University, Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"19169","published-online":{"date-parts":[[2021,6,30]]},"reference":[{"key":"ref0","doi-asserted-by":"crossref","unstructured":"ADAM S, ALMEIDA MF, ASSOUN M, BARUTEAU J, BERNABE SM, BIGOT S, CHAMPION H, DALY A, DASSY M, DAWSON S, ET AL.: Dietary management of urea cycle disorders: European practice. 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