Decreased serum antioxidant capacity in patients with Wilson disease is associated with neurological symptoms

Brůha, Radan; Vı́tek, Libor; Marecek, Z; Pospíšilová, Lenka; Nevšímalová, Soňa; Martásek, Pavel; Petrtýl, J; Urbánek, Petr; Jirásková, Alena; Malíková, Ivana; Haluzı́k, Martin; Ferenci, Péter

doi:10.1007/s10545-011-9422-5

Cited by 31 publications

(18 citation statements)

References 44 publications

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“…Increased serum and CSF copper following penicillamine treatment in WD patient has been reported by Brewer et al (2009). Redistribution of copper from the liver and other viscera to brain has also been reported in experimental animal resulting in reduced GSH and increased MDA levels in basal ganglia and cerebral cortex (Ozcelik and Uzun, 2009;Bruha et al, 2012;Chen, 2012). Brain is more vulnerable to oxidative stress resulting in deterioration of cognitive and motor functions.…”

Section: Discussionmentioning

confidence: 88%

MRI and oxidative stress markers in neurological worsening of Wilson disease following penicillamine

et al. 2015

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Section: Discussionmentioning

confidence: 88%

MRI and oxidative stress markers in neurological worsening of Wilson disease following penicillamine

et al. 2015

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“…Finally, assessment of new as well as existing treatment strategies by prospective randomized clinical trials would be facilitated by the identification of WD biomarkers. 110 …”

Section: Discussionmentioning

confidence: 99%

Wilson's disease and other neurological copper disorders

Bandmann

Weiss

Kaler

2015

The Lancet Neurology

834

534

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Summary The classic copper metabolism disorder, Wilson disease (WD), was first defined in 1912. Both early onset presentations in infancy and late onset manifestations in adults > 70 years are now well recognized. Modern biochemical and genetic prevalence studies suggest that WD may be considerably more common than previously appreciated. Early diagnosis of WD is crucial to ensure that patients can be started on adequate treatment but uncertainty remains about the best possible choice of medication. Direct genetic testing for ATP7B mutations is increasingly available to confirm the clinical diagnosis of WD. WD needs to be differentiated from other conditions that present clinically with hepatolenticular degeneration or share biochemical abnormalities with WD, such as reduced serum cerulo plasmin levels. Disordered copper metabolism is also implied in an increasing number of other neurological conditions, including a subtype of axonal neuropathy due to ATP7A mutations, and the common late-onset neurodegenerative disorders Alzheimer’s disease and Parkinson’s disease.

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“…As the antioxidant material in serum is a mix of components, total antioxidant capacity is generally used as an indicator of the antioxidant capacity of the body, reflecting the sum of all the antioxidant substances present in the serum [11–13]. The antioxidant capacity per unit mass can be obtained by dividing the total antioxidant capacity by the total mass of all substances.…”

Section: Introductionmentioning

confidence: 99%

Determination of the Total Mass of Antioxidant Substances and Antioxidant Capacity per Unit Mass in Serum Using Redox Titration

Zhang

Liu

2014

Bioinorganic Chemistry and Applications

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Objective. Total antioxidant capacity in serum is determined by the total mass of antioxidant substances and the antioxidant capacity per unit mass (average activity). The purpose of this study was to develop a method to determine the mass of antioxidant substances and average activity in human serum. Methods. Specimens of serum were collected from 100 subjects each from two different age groups: over 75 years old and 20–40 years old. The test serum was diluted into a series of concentrations, following which standard oxidation agents (KMnO4 for potassium permanganate method and I2 for iodimetry) were added to each concentration of serum, and the absorbance of the mixture (optical density, OD) was measured. The OD value and logarithm of dilution factor (lgT) at the end of the titration were obtained, from which the lgT could be considered as mass of antioxidant substances (M). Total antioxidant capacity (Ta) was calculated with the equation Ta = 100/(OD1 + 2 ∗ OD2 + 2 ∗ OD3 + 2 ∗ OD4 + OD5), and average activity (A) was calculated as A = Ta/M. Results. The potassium permanganate method generated similar results to the iodimetric method. Compared with the younger group, total antioxidant capacity in the over-75-year age group was found to be significantly reduced, along with a decrease in the mass of antioxidant substances and average activity levels in human serum. Conclusions. The approach described in this paper is suitable for determining the average activity and mass of antioxidant substances in human serum.

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Decreased serum antioxidant capacity in patients with Wilson disease is associated with neurological symptoms

Abstract: Data from our study suggest that the increased oxidative stress contributes significantly to the clinical manifestation of WD; as a lower TAC is associated with the neurological symptoms in WD patients.

Cited by 31 publications

References 44 publications

MRI and oxidative stress markers in neurological worsening of Wilson disease following penicillamine

MRI and oxidative stress markers in neurological worsening of Wilson disease following penicillamine

Wilson's disease and other neurological copper disorders

Determination of the Total Mass of Antioxidant Substances and Antioxidant Capacity per Unit Mass in Serum Using Redox Titration

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