These mutations cause for the deficiency of 3-methylglutaconyl-CoA hydratase which leads to the amalgamation of  3-methylglutaconyl-CoA, 3-methylglutaric acid, and 3-hydroxyisovaleric acid which eventually leads to 3-Methylglutaconic Acuduria Type 1.
Specific genetic alterations cause a shortage of 3-methylglutaconyl-CoA hydratase, causing the formation of 3-methylglutaconyl-CoA, 3-methylglutaric acid, and 3-hydroxyisovaleric acid, which ultimately leads to 3-Methylglutaconic Aciduria Type 1.