Alpha-Aminoadipic Acid (Genova)

Genova: Amino Acids 40 Profile - Plasma

A healthy result should fall into the range 0 - 1.5 µmol/L.

Alpha-aminoadipic acid (α-Aminoadipic acid) is an intermediary metabolite of lysine (primarily) and of tryptophan.

Alpha-aminoadipic acid is elevated in the urine and the most commonly encountered cause is vitamin B6 insufficiency or pyridoxal 5-phosphate dysfunction as a coenzyme for transamination.

Alpha-aminoadipic acid also is a metabolite of yeast/fungi metabolism, and anecdotal evidence supports occasional increases with intestinal dysbiosis as a source.

In Reye’s syndrome, alpha-aminoadipic hyperaminoaciduria occurs together with hyperlysinuria. Rare metabolic and acute alpha-aminoadipic hyperaminoaciduria is documented as due to a hereditary defect in the Alpha-aminoadipic acid transaminase enzyme and in the next enzyme in the sequence, alpha-ketoadipic acid dehydrogenase.

In glutaric acidemia/aciduria there can be notable alpha-aminoadipic hyper-aminoaciduria due to a hereditary weakness of glutaryl CoA dehydrogenase. Riboflavin insufficiency as FAD may provoke or worsen alpha-aminoadipic hyperaminoaciduria (and glutaric acidosis) if the dehydrogenase enzymes are weak, but riboflavin may or may not improve the kinetics of defective dehydrogenases.

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