Glutaric Acid (Glutarate) is endogenously produced in the catabolism of lysine and tryptophan.
- Increased Glutaric acid is associated with secondary carnitine deficiency.
- Glutaryl-CoA (from lysine or tryptophan) normally enters the Krebs cycle via transition to acetyl-CoA.
» Glutaryl-CoA dehydrogenase (GCDH) + glutaryl-CoA + B2 → acetyl-CoA.
» If GCDH is blocked, glutaryl-CoA + carnitine → elevated glutaric acid.
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Elevations of urinary glutaric acid may reflect enzymatic insufficiency requiring vitamin B2 or mitochondrial electron transport dysfunction. Deficiencies of the enzyme glutaryl-CoA dehydrogenase, and multiple acyl-CoA dehydrogenase deficiency (MADD), are well-studied inborn errors of metabolism which result in significant glutaric aciduria. However, milder forms of this rare mitochondrial disorder exist and can result in adult-onset presentations. Late-onset forms can present as atypical beta-oxidation disorders with exercise intolerance, muscle weakness, and CNS dysfunction. In these cases, riboflavin, carnitine, and CoQ10 have been used therapeutically.
Possible causes:
- Low B2
- Inborn error of metabolism
- Associated with CETP genetic polymorphism
Additional investigations:
- Red blood cell B2
- Genetic Diagnostic Testing
Treatment considerations:
- Supplement B2
- Consider mitochondrial support nutrients (e.g. CoQ10)
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