Abstract

Inborn errors of amino acid metabolism include inherited biochemical disorders in which a specific enzyme defect interferes with the normal metabolism of protein. In these disorders as a result of diminished or absent enzyme activity, certain compounds accumulate in the body to toxic levels. Metabolic disturbances can lead to severe cognitive impairment and even death. Phenylketonuria, is the most common amino acid disorder. Dietary restriction prevent the accumulation of a substrate to toxic levels to a certain extend but application of biotechnological methods could provided more promising tools. The main topics discussed are: removal of phenylalanine by activated carbon, by tailored enzymatic hydrolyses of cheese whey, by modified corn cobs as adsorbents and by using enzymatic membrane reactor.

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