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Urinary 2-hydroxy-5-oxoproline, the lactam form of α-ketoglutaramate, is markedly increased in urea cycle disorders

α-Ketoglutaramate (KGM) is the α-keto acid analogue of glutamine, which exists mostly in equilibrium with a lactam form (2-hydroxy-5-oxoproline) under physiological conditions. KGM was identified in human urine and its concentration quantified by gas chromatography/mass spectrometry (GC/MS). The ket...

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Detalhes bibliográficos
Main Authors: Kuhara, Tomiko, Inoue, Yoshito, Ohse, Morimasa, Krasnikov, Boris F., Cooper, Arthur J. L.
Formato: Artigo
Idioma:Inglês
Publicado em: 2011
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3412270/
https://ncbi.nlm.nih.gov/pubmed/21298421
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1007/s00216-011-4688-x
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