Serum concentrations of NSE and S100B in spinocerebellar ataxia type 3/Machado-Joseph disease
10.3969/j.issn.1672-7347.2011.06.006
- VernacularTitle:脊髓小脑性共济失调3型/ Machado-Joseph病血清NSE与S100B浓度的测定
- Author:
Jie ZHOU
;
Lifang LEI
;
Yuting SHI
;
Junling WANG
;
Hong JIANG
;
Lu SHEN
;
Beisha TANG
- Publication Type:Journal Article
- Keywords:
neuron-specific enolase (NSE);
protein S 100 B(S100B);
spinocerebellar ataxia type 3;
Machado-Joseph disease;
biochemical markers
- From:
Journal of Central South University(Medical Sciences)
2011;36(6):504-510
- CountryChina
- Language:Chinese
-
Abstract:
Objective To determine the neuronal damage or loss and gliosis at the cellular level in spinocerebellar ataxia type 3/Machado-Joseph disease(SCA3/MJD), and evaluate the potential use of neuron-specific enolase (NSE) and protein S 100 B(S100B) serum concentrations as biochemical markers. Methods Serum concentrations of NSE and S100B were measured in 102 SCA3/MJD patients and 100 healthy subjects matched by sex and age. The correlations between both markers and age, age of onset, disease duration, CAG repeat size, scores of international cooperative ataxia rating scale(ICARS), and scale for the assessment and rating of ataxia(SARA) were analyzed. Results Compared with the healthy controls, patients with SCA3/MJD had higher NSE serum concentrations [(6.95±2.83)ng/mL vs (4.83±1.70) ng/mL, P<0.05] and higher S100B serum concentrations [(0.07±0.06) ng/mL vs (0.05±0.02) ng/mL, P<0.05]. In the SCA3/MJD patients group, NSE levels presented a positive correlation with age, disease duration, ICARS scores and SARA scores, whereas S100B levels did not correlate with age, age of onset, disease duration, ICARS scores and SARA scores. CAG repeat size did not correlate with the NSE levels and S100B levels in different age groups of SCA3/MJD patients. Conclusion Serum NSE might be a useful marker to monitor disease progression and represent the degree of severity of a certain disease. Elevated S100B serum concentrations in patients compared to healthy controls may suggest an application of this protein as a peripheral marker of brain impairment in SCA3/MJD.
