![]() Acidosis typically occurs when arterial pH falls below 7.35. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. 3-Methyl-2-oxovaleric acid is a keto-acid, which is a subclass of organic acids. The result is a compromise of energy metabolism because of a failure of the malate-aspartate shuttle and a diminished rate of protein synthesis (PMID: 15930465 ). This leads to a depletion of glutamate and a consequent reduction in the concentration of brain glutamine, aspartate, alanine, and other amino acids. In maple syrup urine disease, the brain concentration of branched-chain ketoacids can increase 10- to 20-fold. ![]() If left untreated in older individuals, during times of metabolic crisis, symptoms of the condition include uncharacteristically inappropriate, extreme, or erratic behaviour and moods, hallucinations, anorexia, weight loss, anemia, diarrhea, vomiting, dehydration, lethargy, oscillating hypertonia and hypotonia, ataxia, seizures, hypoglycemia, ketoacidosis, opisthotonus, pancreatitis, rapid neurological decline, and coma. MSUD may also present later depending on the severity of the disease. The symptoms of MSUD often show in infancy and lead to severe brain damage if untreated. MSUD is a metabolic disorder caused by a deficiency of the branched-chain alpha-keto acid dehydrogenase complex (BCKDC), leading to a buildup of the branched-chain amino acids (leucine, isoleucine, and valine) and their toxic by-products (ketoacids) in the blood and urine. Chronically high levels of 3-methyl-2-oxovaleric acid are associated with maple syrup urine disease. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A neurotoxin causes damage to nerve cells and nerve tissues. 3-Methyl-2-oxovaleric acid is a neurotoxin, an acidogen, and a metabotoxin. Enzymes (5) Show 5 proteins Record Informationģ-Methyl-2-oxovaleric acid (CAS: ) is an abnormal metabolite that arises from the incomplete breakdown of branched-chain amino acids.
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