Isobutyryl-coenzyme A dehydrogenase deficiency

Isobutyryl-coenzyme A dehydrogenase deficiency
Other namesIBD deficiency
Valine

Isobutyryl-coenzyme A dehydrogenase deficiency is a rare metabolic disorder in which the body is unable to process certain amino acids properly.

People with this disorder have inadequate levels of an enzyme that helps break down the amino acid valine, resulting in a buildup of valine in the urine, a symptom called valinuria.

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