NDLI: Evaluation of dynamic thiol/disulphide homeostasis as a novel indicator of oxidative stress in maple syrup urine disease patients under treatment

Content Provider	Springer Nature Link
Author	Zubarioglu, Tanyel Kiykim, Ertugrul Cansever, Mehmet Serif Neselioglu, Salim Aktuglu Zeybek, Cigdem Erel, Ozcan
Copyright Year	2016
Abstract	Maple syrup urine disease (MSUD) is a metabolic disorder that is caused by deficiency of branched-chain α-keto acid dehydrogenase complex. Although accumulation of toxic metabolites is associated with neurotoxicity, mechanisms underlying brain damage remain unclear. Aim of this study is to evaluate thiol/disulphide homeostasis as a novel indicator of oxidative stress in MSUD patients under treatment. Twenty patients with MSUD and 20 healthy individuals were included in study. All patients were under regular follow-up and had a good metabolic control. Serum native thiol (−SH), total thiol (−SH + −S-S-), disulphide (−S-S) levels were measured in all subjects. Disulphide/native thiol, disulphide/total thiol and native thiol/total thiol ratios were calculated from these values. Simultaneous blood sampling for plasma quantitative amino acid analysis was performed in both groups. Any significant difference was not observed in -SH, −SH + −S-S-, −S-S levels between two groups. In addition no increase of disulphide/native thiol and disulphide/total thiol ratios was detected in patient group. This study is the first study that evaluates dynamic thiol/disulphide homeostasis as an indicator of oxidative stress in MSUD patients. Among previous studies that were made to determine oxidative stress in treated MSUD patients, this study had the largest sample size also. In recent studies, it was claimed that oxidative stress could be responsible from neurotoxicity even in treated patients. Here, dynamic thiol/disulfide homeostasis status showed that providing good metabolic control in MSUD patients prevent oxidative stress. Under regular follow-up and good compliance with diet, additional antioxidant therapies would possibly not be necessary.
Starting Page	179
Ending Page	184
Page Count	6
File Format	PDF
ISSN	08857490
Journal	Metabolic Brain Disease
Volume Number	32
Issue Number	1
e-ISSN	15737365
Language	English
Publisher	Springer US
Publisher Date	2016-08-18
Publisher Place	New York
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	MSUD Thiol/disulphide homeostasis Oxidative stress Neurotoxicity Neurosciences Neurology Metabolic Diseases Biochemistry Oncology
Content Type	Text
Resource Type	Article
Subject	Biochemistry Neurology (clinical) Cellular and Molecular Neuroscience

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