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Related Experiment Video

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Lentiviral Vector-mediated Gene Therapy of Hepatocytes Ex Vivo for Autologous Transplantation in Swine

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L-carnitine therapy in isovaleric acidemia.

C R Roe, D S Millington, D A Maltby

The Journal of Clinical Investigation

|December 1, 1984

Summary

Isovaleric acidemia treatment involves L-carnitine supplementation. This genetic disorder shows reduced free carnitine, but L-carnitine effectively removes isovaleryl-CoA, preventing hospitalizations.

Area of Science:

Biochemistry
Metabolic Disorders
Genetics

Background:

Isovaleric acidemia (IVA) stems from isovaleryl-coenzyme A dehydrogenase deficiency.
This genetic disorder causes a significant decrease in free carnitine levels in plasma and urine.

Observation:

A novel metabolite, isovalerylcarnitine, was identified using advanced mass spectrometry techniques.
Isovalerylcarnitine is specific to isovaleric acidemia.

Findings:

Supplemental L-carnitine facilitates the removal of isovaleryl-coenzyme A by forming isovalerylcarnitine.
This removal is as effective as glycine conjugation, which forms isovalerylglycine.
L-carnitine monotherapy increases the excretion of isovalerylcarnitine and hippurate while decreasing isovalerylglycine.

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Implications:

L-carnitine supplementation alone can be an effective treatment strategy for isovaleric acidemia.
This approach may help prevent hospitalizations in patients with this metabolic disorder.