{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"omics_type":["Unknown"],"volume":["21"],"submitter":["Kuhn S"],"pubmed_abstract":["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 "],"journal":["Computational and structural biotechnology journal"],"pagination":["1236-1248"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC9932296"],"repository":["biostudies-literature"],"pubmed_title":["The 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."],"pmcid":["PMC9932296"],"pubmed_authors":["Sass JO","van der Sluis R","Williams ME","Dercksen M","Kuhn S"],"additional_accession":[]},"is_claimable":false,"name":"The 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.","description":"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 ","dates":{"release":"2023-01-01T00:00:00Z","publication":"2023","modification":"2026-05-29T08:26:20.86Z","creation":"2024-11-06T12:50:30.253Z"},"accession":"S-EPMC9932296","cross_references":{"pubmed":["36817957"],"doi":["10.1016/j.csbj.2023.01.041"]}}