Literature DB >> 6101846

Enzymatic control of phenylalanine intake in phenylketonuria.

J A Hoskins, G Jack, H E Wade, R J Peiris, E C Wright, D J Starr, J Stern.   

Abstract

The plant enzyme phenylalanine ammonia lyase (PAL) will survive in the gut for long enough to deplete the phenylalanine derived from food protein and so reduce the rise in blood phenylalanine that otherwise occurs after a protein meal. This effect has been demonstrated in healthy adults and phenylketonuric (PKU) patients. When the enzyme was given to an untreated PKU patient for 12 consecutive days (three doses a day after food) his blood phenylalanine levels were reduced on average by a quarter. This enzyme preparation may have a place in the treatment of PKU.

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Year:  1980        PMID: 6101846     DOI: 10.1016/s0140-6736(80)90944-7

Source DB:  PubMed          Journal:  Lancet        ISSN: 0140-6736            Impact factor:   79.321


  19 in total

Review 1.  Phenylketonuria: old disease, new approach to treatment.

Authors:  H L Levy
Journal:  Proc Natl Acad Sci U S A       Date:  1999-03-02       Impact factor: 11.205

2.  Strain Improvement of Rhodotorula graminis for Production of a Novel l-Phenylalanine Ammonia-Lyase.

Authors:  S A Orndorff; N Costantino; D Stewart; D R Durham
Journal:  Appl Environ Microbiol       Date:  1988-04       Impact factor: 4.792

3.  The effect of proteinases on phenylalanine ammonia-lyase from the yeast Rhodotorula glutinis.

Authors:  H J Gilbert; G W Jack
Journal:  Biochem J       Date:  1981-12-01       Impact factor: 3.857

4.  Synthesis and degradation of phenylalanine ammonia-lyase of Rhodosporidium toruloides.

Authors:  H J Gilbert; M Tully
Journal:  J Bacteriol       Date:  1982-05       Impact factor: 3.490

5.  A different approach to treatment of phenylketonuria: phenylalanine degradation with recombinant phenylalanine ammonia lyase.

Authors:  C N Sarkissian; Z Shao; F Blain; R Peevers; H Su; R Heft; T M Chang; C R Scriver
Journal:  Proc Natl Acad Sci U S A       Date:  1999-03-02       Impact factor: 11.205

Review 6.  Up to date knowledge on different treatment strategies for phenylketonuria.

Authors:  Amaya Bélanger-Quintana; Alberto Burlina; Cary O Harding; Ania C Muntau
Journal:  Mol Genet Metab       Date:  2011-08-16       Impact factor: 4.797

7.  Control of synthesis of functional mRNA coding for phenylalanine ammonia-lyase from Rhodosporidium toruloides.

Authors:  H J Gilbert; J R Stephenson; M Tully
Journal:  J Bacteriol       Date:  1983-03       Impact factor: 3.490

8.  Preclinical evaluation of multiple species of PEGylated recombinant phenylalanine ammonia lyase for the treatment of phenylketonuria.

Authors:  Christineh N Sarkissian; Alejandra Gámez; Lin Wang; Marilyse Charbonneau; Paul Fitzpatrick; Jeffrey F Lemontt; Bin Zhao; Michael Vellard; Sean M Bell; Carroll Henschell; Amy Lambert; Laurie Tsuruda; Raymond C Stevens; Charles R Scriver
Journal:  Proc Natl Acad Sci U S A       Date:  2008-12-18       Impact factor: 11.205

Review 9.  Phenylketonuria due to phenylalanine hydroxylase deficiency: an unfolding story. Medical Research Council Working Party on Phenylketonuria.

Authors: 
Journal:  BMJ       Date:  1993-01-09

10.  Expression in E. coli of the gene encoding phenylalanine ammonia-lyase from Rhodosporidium toruloides.

Authors:  H Orum; O F Rasmussen
Journal:  Appl Microbiol Biotechnol       Date:  1992-03       Impact factor: 4.813

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