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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
4
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pubmed:dateCreated |
1981-4-21
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pubmed:abstractText |
Urinary excretion of the organic acids in patients with type I and III glycogenosis was investigated. In all patients with type I glycogenosis, urinary alpha-ketoglutarate concentration ws about 10 times the normal value. alpha-Ketoglutaric aciduria was not improved by the acute or prolonged administration of a large dose of factors for pyruvate- and alpha-ketoglutarate dehydrogenase complex. On the other hand, the level of alpha-ketoglutarate in the urine from type I patients decreased in conjunction with the decrease of plasma lactate and pyruvate concentration after repeated oral glucose loading. Oral citrate loading brought an increased excretion of alpha-ketoglutarate in type I glycogenosis. It is possible that alpha-ketoglutarate dehydrogenase in the rate-limiting step in tricarboxylic acid cycle and in patients with glycogenosis type I, the excessive excretion of alpha-ketoglutarate may be caused by the limited activity of alpha-ketoglutarate dehydrogenase with excessive substrate.
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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/Citrates,
http://linkedlifedata.com/resource/pubmed/chemical/Glucose,
http://linkedlifedata.com/resource/pubmed/chemical/Ketoglutaric Acids,
http://linkedlifedata.com/resource/pubmed/chemical/Lactates,
http://linkedlifedata.com/resource/pubmed/chemical/Pyruvates
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pubmed:status |
MEDLINE
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pubmed:month |
Aug
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pubmed:issn |
0040-8727
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:volume |
131
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
347-53
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pubmed:dateRevised |
2006-11-15
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pubmed:meshHeading |
pubmed-meshheading:6936873-Adolescent,
pubmed-meshheading:6936873-Child,
pubmed-meshheading:6936873-Child, Preschool,
pubmed-meshheading:6936873-Citrates,
pubmed-meshheading:6936873-Female,
pubmed-meshheading:6936873-Glucose,
pubmed-meshheading:6936873-Glycogen Storage Disease Type I,
pubmed-meshheading:6936873-Glycogen Storage Disease Type III,
pubmed-meshheading:6936873-Humans,
pubmed-meshheading:6936873-Ketoglutaric Acids,
pubmed-meshheading:6936873-Lactates,
pubmed-meshheading:6936873-Male,
pubmed-meshheading:6936873-Pyruvates
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pubmed:year |
1980
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pubmed:articleTitle |
Studies on alpha-ketoglutaric aciduria in type I glycogenosis.
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pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
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