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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
7
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pubmed:dateCreated |
1992-11-19
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pubmed:abstractText |
Many enzymes that require pyridoxal 5'-phosphate (PLP), a coenzyme from a vitamin B6, are involved in amino acid metabolism. B6 dependency syndromes are defined as a group of metabolic disorders which are prevented or alleviated by non-physiologically large doses of vitamin B6, and, hence, they are tacitly accounted for by some structural alteration in a responsible B6-dependent enzyme such as a decrease on the affinity for PLP as compared to the normal. In this article, the mode of binding the coenzyme is exemplified by the case of aspartate aminotransferase, a typical B6-dependent enzyme whose three-dimensional structure is known, and, several B6 dependency syndromes are briefly reviewed. Among these syndromes, the molecular basis of only gyrate atrophy has recently been defined by the identification of a mutation in the relevant enzyme, ornithine aminotransferase.
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pubmed:language |
jpn
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:chemical |
http://linkedlifedata.com/resource/pubmed/chemical/Aspartate Aminotransferases,
http://linkedlifedata.com/resource/pubmed/chemical/Cystathionine,
http://linkedlifedata.com/resource/pubmed/chemical/Ornithine,
http://linkedlifedata.com/resource/pubmed/chemical/Pyridoxal Phosphate,
http://linkedlifedata.com/resource/pubmed/chemical/Pyridoxine
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pubmed:status |
MEDLINE
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pubmed:month |
Jul
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pubmed:issn |
0047-1852
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:volume |
50
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
1581-6
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pubmed:dateRevised |
2011-7-27
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pubmed:meshHeading |
pubmed-meshheading:1404887-Amino Acid Metabolism, Inborn Errors,
pubmed-meshheading:1404887-Aspartate Aminotransferases,
pubmed-meshheading:1404887-Cystathionine,
pubmed-meshheading:1404887-Homocystinuria,
pubmed-meshheading:1404887-Humans,
pubmed-meshheading:1404887-Hyperoxaluria,
pubmed-meshheading:1404887-Ornithine,
pubmed-meshheading:1404887-Pyridoxal Phosphate,
pubmed-meshheading:1404887-Pyridoxine
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pubmed:year |
1992
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pubmed:articleTitle |
[Vitamin B6 dependency syndrome].
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pubmed:affiliation |
Department of Biochemistry, Kumamoto University Medical School.
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pubmed:publicationType |
Journal Article,
English Abstract
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