Urinary biomarkers of oxidative damage in Maple syrup urine disease: The l-carnitine role

Gilian Guerreiro,Caroline Paula Mescka,Angela Sitta,Bruna Donida,Desirèe Marchetti, Tatiane Hammerschmidt,Jessica Faverzani,Daniella de Moura Coelho,Moacir Wajner,Carlos Severo Dutra-Filho,Carmen Regla Vargas

International Journal of Developmental Neuroscience（2015）

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摘要

•Maple syrup urine disease (MSUD) patients have l-carnitine (l-car) deficiency.•There is damage to proteins and lipids in urine and a deficient antioxidant capacity in MSUD patients.•l-car supplementation could be involved in the prevention of this oxidative damage.

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关键词

ABTS,BCAA,BCKAD,Di-tyr,ELISA,GC/MS,HPLC,HRP,KIC,Leu,l-car,LC/MS/MS,MRM,MSUD

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