| An extracorporeal hollow-fiber reactor for phenylketonuria using immobilized phenylalanine ammonia lyase. | |
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MedLine Citation:
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PMID: 3956347 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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C Larue; A Munnich; C Charpentier; J M Saudubray; J Frézal; M H Rémy; C Rivat |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Developmental pharmacology and therapeutics Volume: 9 ISSN: 0379-8305 ISO Abbreviation: Dev Pharmacol Ther Publication Date: 1986 |
Date Detail:
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Created Date: 1986-05-06 Completed Date: 1986-05-06 Revised Date: 2009-11-19 |
Medline Journal Info:
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Nlm Unique ID: 8003947 Medline TA: Dev Pharmacol Ther Country: SWITZERLAND |
Other Details:
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Languages: eng Pagination: 73-81 Citation Subset: IM |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Ammonia-Lyases* Enzymes, Immobilized* Extracorporeal Circulation / instrumentation* Humans Phenylalanine / blood Phenylalanine Ammonia-Lyase* Phenylketonurias / blood, therapy* Renal Dialysis Time Factors |
| Chemical | |
Reg. No./Substance:
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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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