Fig. 1From: Clinical improvements after treatment with a low-valine and low-fat diet in a pediatric patient with enoyl-CoA hydratase, short chain 1 (ECHS1) deficiencyIllustration of the short-chain fatty acid, odd-chain fatty acid and valine catabolic pathways, showing the formation of certain metabolites as a result of short-chain enoyl-CoA hydratase 1 (ECHS1) deficiency. The concept that 2,3-dihydroxy-2-methylbutyric acid originates from acryloyl-CoA was indirectly inferred based on Peters et al. [6]Back to article page