Glutaric Acidemia Type I (GA1) is a rare neurometabolic disorder caused by a deficiency in the enzyme glutaryl-CoA dehydrogenase (GCDH). This condition leads to the accumulation of toxic ...
methylmalonic acidemia; MADD, multiple acyl-CoA dehydrogenased deficiency (GAII, glutaric acidemia, type II); PEPCK, phosphoenolpyruvate carbosykinase deficiency; PPA, propionic acidemia ...
Isovaleric Acidemia is treated with L-leucine restriction and supplemental glycine. Added glycine augments conversion of isovaleric acid (IVA) to isovalerylglycine (IVG) through glycine-N-acylase.
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