Glutaric aciduria type I and kynurenine pathway metabolites: a modified hypothesis.

doi:10.1023/B:BOLI.0000045767.42193.97

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Article

Glutaric aciduria type I and kynurenine pathway metabolites: a modified hypothesis.

Journal of inherited metabolic disease - 1 Jan 2004

Varadkar S, Surtees R

Abstract excerpt

Glutaric aciduria type I is an inborn error of organic acid metabolism that demonstrates a particular temporal vulnerability (acute encephalopathic episodes in infancy) and a spatial vulnerability (acute striatal necrosis, focused on the putamen). Excitotoxic mechanisms involving 3-hydroxyglutaric acid as the major neurotoxin have been suggested. This paper proposes a role for metabolites of the kynurenine...

Topics

Amino Acid Metabolism, Inborn Errors
Animals
Glutarates
Humans
Kynurenine
Phenotype

Source

Open in PubMed

Identifiers

PMID: 15505390
DOI: 10.1023/B:BOLI.0000045767.42193.97

Provenance

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