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On the origin of 3-methylglutaconic acid in disorders of mitochondrial energy metabolism
3-methylglutaconic acid (3MGA)-uria occurs in numerous inborn errors of metabolism (IEM) associated with compromised mitochondrial energy metabolism. This organic acid arises from thioester cleavage of 3-methylglutaconyl CoA (3MG CoA), an intermediate in leucine catabolism. In individuals harboring...
Enregistré dans:
| Publié dans: | J Inherit Metab Dis |
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| Auteurs principaux: | , |
| Format: | Artigo |
| Langue: | Inglês |
| Publié: |
2016
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| Sujets: | |
| Accès en ligne: | https://ncbi.nlm.nih.gov/pmc/articles/PMC4988875/ https://ncbi.nlm.nih.gov/pubmed/27091556 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1007/s10545-016-9933-1 |
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