Abstract:
:Phenylketonuria (PKU) is an autosomal recessive genetic disease caused by the defects in the phenylalanine hydroxylase (PAH) gene. Individuals homozygous for defective PAH alleles show elevated levels of systemic phenylalanine and should be under strict dietary control to reduce the risk of neuronal damage associated with high levels of plasma phenylalanine. Researchers predict that plant phenylalanine ammonia-lyase (PAL), which converts phenylalanine to nontoxic t-cinnamic acid, will be an effective therapeutic enzyme for the treatment of PKU. The problems of this potential enzyme therapy have been the low stability in the circulation and the antigenicity of the plant enzyme. Recombinant PAL originated from parsley (Petroselinum crispum) chemically conjugated with activated PEG2 [2,4-bis(O-methoxypolyethyleneglycol)-6-chloro-s-triazine] showed greatly enhanced stability in the circulation and was effective in reducing the plasma concentration of phenylalanine in the circulation of mice. PEG-PAL conjugate will be an effective therapeutic enzyme for the treatment of PKU.
journal_name
Amino Acidsjournal_title
Amino acidsauthors
Ikeda K,Schiltz E,Fujii T,Takahashi M,Mitsui K,Kodera Y,Matsushima A,Inada Y,Schulz GE,Nishimura Hdoi
10.1007/s00726-005-0218-5subject
Has Abstractpub_date
2005-11-01 00:00:00pages
283-7issue
3eissn
0939-4451issn
1438-2199journal_volume
29pub_type
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