Optimal Result:
0 - 2 umol/L.
Amino Acid Analysis, Plasma - Amino Acid analysis is necessary for the diagnosis of a variety of inborn errors of metabolism. These include, but are not limited to, phenylketonuria, tyrosinemia, citrullinemia, non-ketotic hyperglycinemia, maple syrup urine disease, and homocystinuria. The assay is also key for the continued monitoring of treatment plans for these disorders and useful for assessing nutritional status of patients.
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.
Lab Results Explained and Tracked
What does it mean if your Alpha Aminoadipic Acid result is too high?
Elevated levels of Alpha Aminoadipic Acid in plasma may be associated with certain inborn errors of amino acid metabolism. These metabolic disorders are caused by genetic defects that affect the synthesis, catabolism, or transport of amino acids, leading to a diverse class of diseases.
Inborn errors of amino acid metabolism can manifest in various ways and have different clinical presentations. For example, some well-known disorders in this category include Phenylketonuria (PKU), Lysinuric Protein Intolerance (LPI), and Homocystinuria due to Cystathionine Beta-Synthase (CBS) deficiency. Each of these conditions has distinct pathophysiological features and clinical implications.
- Phenylketonuria is characterized by defects in phenylalanine hydroxylase, leading to the accumulation of phenylalanine.
- Lysinuric Protein Intolerance results from defects in the transport of certain amino acids, leading to malabsorption and secondary urea cycle defects.
- Homocystinuria is caused by a defect in cystathionine β-synthase, leading to the accumulation of methionine and homocysteine.
It's important to note that while these conditions are examples of inborn errors of amino acid metabolism, they may not be directly related to elevated Alpha Aminoadipic Acid levels. The specific link between such elevated levels and particular metabolic disorders would require further clinical investigation and correlation with other clinical findings.
Elevated levels of Alpha-Aminoadipic Acid in plasma can also be caused by various conditions, including vitamin B6 insufficiency or pyridoxal 5-phosphate dysfunction. Vitamin B6 is crucial for transaminase enzymes, which convert one amino acid to another and help them enter the Krebs cycle. Therefore, a deficiency in vitamin B6 can lead to an accumulation of Alpha-Aminoadipic Acid.
A potential treatment approach for elevated Alpha-Aminoadipic Acid levels involves supplementing with vitamin B6 and α-KG (alpha-Ketoglutarate). This supplementation aims to facilitate the transamination conversion of Alpha-Aminoadipic Acid to Alpha-Ketoadipic Acid. However, it's important to note that the effectiveness and appropriateness of this treatment can vary depending on the underlying cause and individual health circumstances.
Additionally, there are instances, such as in Pyridoxine-Dependent Epilepsy (PDE), where patients are already on daily pyridoxine supplements. In such cases, treatment approaches can be more complex and tailored to the specific disorder and patient needs.
Given the complexity and potential variability in underlying causes, it's essential to consult a healthcare provider for an accurate diagnosis and appropriate treatment plan tailored to the individual's specific condition and overall health profile.
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