Source:http://linkedlifedata.com/resource/pubmed/id/10083729
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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
2
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pubmed:dateCreated |
1999-4-7
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pubmed:abstractText |
Alkaptonuria is a rare autosomal recessive disorder characterized by homogentisic aciduria, ochronosis, and arthritis. Although a deficiency of homogentisic acid 1,2-dioxygenase has recently been confirmed at the molecular level, no effective treatment regimen has yet been developed for this disorder. In the present study, 2(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC), a potent inhibitor of p-hydroxyphenylpyruvate dioxygenase (which catalyzes the formation of homogentisic acid from p-hydroxyphenylpyruvic acid) was adopted as a possible therapeutic agent for alkaptonuria. NTBC dose-dependently reduced the urinary output of homogentisic acid in a murine model of alkaptonuria that had been created with ethylnitrosourea. These findings suggest that NTBC may be the first potent pharmacotherapeutic agent for alkaptonuria.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:chemical |
http://linkedlifedata.com/resource/pubmed/chemical/4-Hydroxyphenylpyruvate Dioxygenase,
http://linkedlifedata.com/resource/pubmed/chemical/Cyclohexanones,
http://linkedlifedata.com/resource/pubmed/chemical/Enzyme Inhibitors,
http://linkedlifedata.com/resource/pubmed/chemical/Homogentisic Acid,
http://linkedlifedata.com/resource/pubmed/chemical/Nitrobenzoates,
http://linkedlifedata.com/resource/pubmed/chemical/nitisinone
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pubmed:status |
MEDLINE
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pubmed:issn |
1434-5161
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:volume |
44
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
79-84
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pubmed:dateRevised |
2003-11-14
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pubmed:meshHeading |
pubmed-meshheading:10083729-4-Hydroxyphenylpyruvate Dioxygenase,
pubmed-meshheading:10083729-Alkaptonuria,
pubmed-meshheading:10083729-Animals,
pubmed-meshheading:10083729-Cyclohexanones,
pubmed-meshheading:10083729-Disease Models, Animal,
pubmed-meshheading:10083729-Enzyme Inhibitors,
pubmed-meshheading:10083729-Homogentisic Acid,
pubmed-meshheading:10083729-Mice,
pubmed-meshheading:10083729-Mice, Mutant Strains,
pubmed-meshheading:10083729-Nitrobenzoates
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pubmed:year |
1999
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pubmed:articleTitle |
A novel therapeutic trial of homogentisic aciduria in a murine model of alkaptonuria.
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pubmed:affiliation |
Department of Clinical Genetics, Kyushu University, Beppu, Japan. dsuzuki@kich.itami.hyogo.jp
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pubmed:publicationType |
Journal Article
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