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KMID : 0857020060210010369
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Kosin Medical Journal
2006 Volume.21 No. 1 p.369 ~ p.374
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Enzyme therapy for phenylketonuria future directions
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Kim Woo-Mi
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Abstract
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The dietary therapy for phenylketonuria (PKU) is unpalatable and ineffective in controlling systemic phenylalanine (Phe) levels during pregnancy. Alternative therapies are currently being investigated, particularly ones that break down Phe. This review underscores the progress made in enzyme replacement therapy for PKU. Two modalities are discussed, the enzymes phenylalanine hydroxylase (PAH) and phenylalanine ammonia-lyase (PAL). Developing stable and functional forms of both enzymes have proven difficult, but recent success in producing PEG-modified form of active and stable PAH shows promise. In addition, microencapsulation (ENC) could partially protect proteolysis and gastric acidity. If the immunologic problems can be overcome by PEGylation, and the activity of PEGylated enzyme can be protected by additional encapsulation, it may provide a new prospect for both the oral and parenteral enzyme therapies in PKU.
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KEYWORD
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phenylketonuria, phenylalanine ammonia-lyase, PEGylation, microencapsulation
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