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Branched-chain amino acid metabolism: from rare Mendelian diseases to more common disorders.
Burrage LC, Nagamani SC, Campeau PM, Lee BH. Burrage LC, et al. Hum Mol Genet. 2014 Sep 15;23(R1):R1-8. doi: 10.1093/hmg/ddu123. Epub 2014 Mar 20. Hum Mol Genet. 2014. PMID: 24651065 Free PMC article. Review.
Branched-chain amino acid (BCAA) metabolism plays a central role in the pathophysiology of both rare inborn errors of metabolism and the more common multifactorial diseases. Although deficiency of the branched-chain ketoacid dehydrogen …
Branched-chain amino acid (BCAA) metabolism plays a central role in the pathophysiology of both rare inborn errors of m …
Lowered concentrations of branched-chain amino acids result in impaired growth and neurological problems: insights from a branched-chain alpha-keto acid dehydrogenase complex kinase-deficient mouse model.
Watford M. Watford M. Nutr Rev. 2007 Apr;65(4):167-72. doi: 10.1111/j.1753-4887.2007.tb00296.x. Nutr Rev. 2007. PMID: 17503711 Review.
Excess circulating levels of branched-chain amino acids (BCAA), as seen in maple syrup urine disease, result in severe neuropathology. A new mouse model, deficient in the kinase that controls BCAA catabolism, shows that very low circulating levels of B …
Excess circulating levels of branched-chain amino acids (BCAA), as seen in maple syrup urine disease, result in severe neuropa …
L-carnitine supplementation as a potential antioxidant therapy for inherited neurometabolic disorders.
Ribas GS, Vargas CR, Wajner M. Ribas GS, et al. Gene. 2014 Jan 10;533(2):469-76. doi: 10.1016/j.gene.2013.10.017. Epub 2013 Oct 19. Gene. 2014. PMID: 24148561 Review.
Thus, l-carnitine supplementation may be useful not only to prevent tissue deficiency of this element, but also to avoid oxidative damage secondary to increased production of reactive species in these diseases. ...
Thus, l-carnitine supplementation may be useful not only to prevent tissue deficiency of this element, but also to avoid oxidative da …