biostudies-literatureUnknown21Kuhn SIsovaleric acidemia (IVA), due to isovaleryl-CoA dehydrogenase (IVD) deficiency, results in the accumulation of isovaleryl-CoA, isovaleric acid and secondary metabolites. The increase in these metabolites decreases mitochondrial energy production and increases oxidative stress. This contributes to the neuropathological features of IVA. A general assumption in the literature exists that glycine <i>N</i>-acyltransferase (GLYAT) plays a role in alleviating the symptoms experienced by IVA patients through the formation of <i>N</i>-isovalerylglycine. GLYAT forms part of the phase II glycine conjugation pathway in the liver and detoxifies excess acyl-CoA's namely benzoyl-CoA. However, very few studies support GLYAT as the enzyme that conjugates isovaleryl-CoA to glycine. Furthermore, GLYATL1, a Computational and structural biotechnology journal1236-1248https://www.ebi.ac.uk/biostudies/studies/S-EPMC9932296biostudies-literatureThe glycine <i>N</i>-acyltransferases, GLYAT and GLYATL1, contribute to the detoxification of isovaleryl-CoA - an <i>in-silico</i> and <i>in vitro</i> validation.PMC9932296Sass JOvan der Sluis RWilliams MEDercksen MKuhn SfalseThe glycine <i>N</i>-acyltransferases, GLYAT and GLYATL1, contribute to the detoxification of isovaleryl-CoA - an <i>in-silico</i> and <i>in vitro</i> validation.Isovaleric acidemia (IVA), due to isovaleryl-CoA dehydrogenase (IVD) deficiency, results in the accumulation of isovaleryl-CoA, isovaleric acid and secondary metabolites. The increase in these metabolites decreases mitochondrial energy production and increases oxidative stress. This contributes to the neuropathological features of IVA. A general assumption in the literature exists that glycine <i>N</i>-acyltransferase (GLYAT) plays a role in alleviating the symptoms experienced by IVA patients through the formation of <i>N</i>-isovalerylglycine. GLYAT forms part of the phase II glycine conjugation pathway in the liver and detoxifies excess acyl-CoA's namely benzoyl-CoA. However, very few studies support GLYAT as the enzyme that conjugates isovaleryl-CoA to glycine. Furthermore, GLYATL1, a 2023-01-01T00:00:00Z20232026-05-29T08:26:20.86Z2024-11-06T12:50:30.253ZS-EPMC99322963681795710.1016/j.csbj.2023.01.041