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Maple Syrup Urine Disease

Maple Syrup Urinal

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Maple syrup urine disease, also called branched-chain ketoaciduria, is an autosomal recessive metabolic disorder caused by a deficiency of the alpha ketoacid dehydrogenase complex. This complex of enzymes is responsible for the degradation of branched chain amino acids including isoleucine, leucine, and valine. Defects in this enzyme cause the presence of sweet-smelling urine, similar to that of maple syrup leading to the name maple syrup urine disease. Infants with this metabolic disorder initially seem healthy at birth but if left untreated can suffer from seizures, CNS defects, and intellectual disability. Other nonspecific symptoms include poor feeding, vomiting, dehydration, lethargy and hypotonia. Treatment includes diet with minimal levels of branched chain amino acids necessary for metabolic functions.

7 KEY FACTS

Maple syrup urine disease, also called branched-chain ketoaciduria, is a metabolic disorder caused by a deficiency of the alpha ketoacid dehydrogenase complex. This enzyme complex structure is analogous to alpha-ketoglutarate dehydrogenase and pyruvate dehydrogenase, requiring the same cofactors thiamine diphosphate, FAD, NAD, lipoate, and coenzyme A. Alpha ketoacid dehydrogenase is responsible for degradation of branched-chain amino acids.

Alpha ketoacid dehydrogenase is responsible for the degradation of branched-chain amino acids isoleucine, leucine, and valine.

Leucine is a branched-chain amino acid that accumulates in the blood and urine in maple syrup urine disease.

Isoleucine is a branched-chain amino acid that accumulates in the blood and urine in maple syrup urine disease.

Valine is a branched-chain amino acid that accumulates in the blood and urine in maple syrup urine disease.

A seizure is defined as a transient episode of abnormal, excessive neuronal activity. Seizures can be a symptom of maple syrup urine disease.

Infants with this metabolic disorder initially seem healthy at birth but, if left untreated, can suffer from CNS defects and intellectual disability.

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