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Indication: | AMMOBULA is used to Treat Urea cycle disorders (UCDs) (UCDs) are inborn errors of metabolism resulting from defects in one of the enzymes or transporter molecules involved in the hepatic removal of ammonia from the bloodstream. Removal of ammonia from the bloodstream normally occurs via its conversion to urea, which is then excreted by the kidneys. Consequently, urea cycle disorders lead to an accumulation of ammonia. Ammonia is extremely toxic, particularly to the central nervous system. Newborns with severe mutations in any one of the first four enzymes of the urea cycle can become catastrophically ill within 36 to 48 hours of birth despite appearing normal at birth. |
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AMMOBULA is a nitrogen-scavenging agent used in the treatment of urea cycle disorders (UCDs). It works by converting to phenylacetic acid (PAA), which then conjugates with glutamine to form phenylacetylglutamine (PAGN), which is excreted in the urine, thus reducing ammonia levels. NaPBA is also known to affect branched-chain amino acid (BCAA) metabolism. |