Glutaric Aciduria Type I - NORD (National Organization for.
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Glutaric aciduria type I (GA I) is a recessive disorder caused by a deficiency of glutaryl-CoA dehydrogenase (GCDH). The biochemical hallmark of the disease is the accumulation of glutaric acid.
Glutaric Acid result calculator insert the value from you Glutaric Acid test result. Result is good! Result is at risk! Result needs work! What does it mean if your Glutaric Acid result is too high? Elevation indicates riboflavin (vitamin B2) deficiency. Other possible factors include fatty acid oxidation defects, and metabolic effects of valproic acid (Depakene), or celiac disease. The.
Nutrition Management of Glutaric Acidemia Type 1. Authors; Authors and affiliations; Laurie E. Bernstein; Chapter. 1.5k Downloads; Abstract. Glutaric acidemia type 1 (GA-1) is an autosomal recessive disorder of lysine, hydroxylysine, and tryptophan metabolism. A defect of glutaryl-CoA dehydrogenase results in the accumulation of 3-hydroxyglutaric acid and glutaric acid. Nutrition management of.
What is Glutaric Aciduria Type 1? Glutaric Aciduria Type 1 (GA-1) is an inherited, genetic disorder. This disorder can also be called Glutaric Acidemia Type 1. GA-1 occurs when there is a defect in an enzyme that helps break down protein from food. The name of the enzyme is glutaryl-CoA dehydrogenase (GCDH). Protein is made up of smaller units called amino acids. Amino acids are important to.
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Chronically high levels of glutaric acid are associated with at least three inborn errors of metabolism, including glutaric aciduria type I, malonyl-CoA decarboxylase deficiency, and glutaric aciduria type III. Glutaric aciduria type I (glutaric acidemia type I, glutaryl-CoA dehydrogenase deficiency, GA1, or GAT1) is an inherited disorder in which the body is unable to completely break down.