Document Detail


An extracorporeal hollow-fiber reactor for phenylketonuria using immobilized phenylalanine ammonia lyase.
MedLine Citation:
PMID:  3956347     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
A hollow-fiber hemodialyzer with immobilized phenylalanine ammonia lyase was tested in vitro for depletion of blood phenylalanine in a recirculating system. A sustained reduction of phenylalanine was obtained in less than 1 h. The product of phenylalanine deamination, trans-cinnamic acid, is a nontoxic compound metabolized to benzoic acid by the liver and eliminated in the urine as hippuric acid. As a model, this reactor may be relevant not only for the short-term management of hyperphenylalaninemia (particularly in pregnant phenylketonuric mothers), but for other metabolic diseases as well, provided that a biocatalyst effective on the accumulating substance is available.
Authors:
C Larue; A Munnich; C Charpentier; J M Saudubray; J Frézal; M H Rémy; C Rivat
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Developmental pharmacology and therapeutics     Volume:  9     ISSN:  0379-8305     ISO Abbreviation:  Dev Pharmacol Ther     Publication Date:  1986  
Date Detail:
Created Date:  1986-05-06     Completed Date:  1986-05-06     Revised Date:  2009-11-19    
Medline Journal Info:
Nlm Unique ID:  8003947     Medline TA:  Dev Pharmacol Ther     Country:  SWITZERLAND    
Other Details:
Languages:  eng     Pagination:  73-81     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
Ammonia-Lyases*
Enzymes, Immobilized*
Extracorporeal Circulation / instrumentation*
Humans
Phenylalanine / blood
Phenylalanine Ammonia-Lyase*
Phenylketonurias / blood,  therapy*
Renal Dialysis
Time Factors
Chemical
Reg. No./Substance:
0/Enzymes, Immobilized; 63-91-2/Phenylalanine; EC 4.3.1.-/Ammonia-Lyases; EC 4.3.1.24/Phenylalanine Ammonia-Lyase

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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