Alpha-aminoadipic acid (a-Aminoadipic acid) is an intermediary metabolite of lysine (primarily) and of tryptophan.
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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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.
B6 is needed by the transaminase enzymes, which convert one amino acid to another, and thus facilitate feeding them into the Krebs cycle. Possible inhibition of lysine metabolism and lowered amine group transfer in the tissues.
Possible treatment:
Supplement vitamin B6 and α-KG to facilitate the transamination conversion of α-aminoadipic to α-ketoadipic acid.
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1-Methylhistidine (Plasma), 3-Methylhistidine (Plasma), Alanine (Plasma), Alpha-Amino-n-butyric acid (Plasma), Alpha-Aminoadipic Acid (Plasma), Alpha-ANB/Leucine, Anserine (Plasma), Arginine (Plasma), Asparagine (Plasma), Aspartic Acid (Plasma), Beta-Alanine, Beta-Aminoisobutyric Acid (Plasma), Carnosine (Plasma), Citrulline, Cystathionine (Plasma), Cystine, Ethanolamine (Plasma), g-Aminobutyric Acid (Plasma), Glutamic Acid (Plasma), Glutamic Acid/Glutamine, Glutamine (Plasma), Glycine (Plasma), Histidine (Plasma), Homocysteine (Genova), Homocystine (Genova), Hydroxylysine (Genova), Hydroxyproline (Genova), Hydroxyproline/Proline (Genova), Isoleucine (Plasma), Leucine (Plasma), Lysine (Plasma), Methionine (Plasma), Ornithine (Genova), Phenylalanine (Plasma), Phenylalanine/Tyrosine (Genova), Phosphoethanolamine (Plasma), Phosphoserine (Plasma), Proline (Plasma), Sarcosine (Plasma), Serine (Plasma), Taurine (Plasma), Threonine (Plasma), Tryptophan (Plasma), Tryptophan/LNAA (Genova), Tyrosine (Plasma), Valine (Plasma)
