Urea Cycle Disorders (UCD)

Mitochondrion

The urea cycle is a vital metabolic process in the liver through which the body eliminates ammonium, which is formed when proteins are degraded. The process involves a series of enzyme reactions, resulting in the end product urea, which is secreted through the urine. In rare cases, children may inherit one or more deficient genes for a urea cycle enzyme from their parents.  Malfunction in any of the enzymes leads to accumulation of excess ammonium in the body, which left untreated will cause irreversible brain damage, coma and ultimately death.