title: Serum concentrations of NSE and S100B in spinocerebellar ataxia type 3/Machado-Joseph disease.
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.
  
  
  CONCLUSIONS
  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.
authors: Department of Neurology, Xiangya Hospital, Central South University, Changsha 410008, China.
authors: Jie
authors: J
authors: Zhou
authors: Lifang
authors: L
authors: Lei
authors: Yuting
authors: Y
authors: Shi
authors: Junling
authors: J
authors: Wang
authors: Hong
authors: H
authors: Jiang
authors: Lu
authors: L
authors: Shen
authors: Beisha
authors: B
authors: Tang
mesh_terms: D000328:Adult; D015415:Biomarkers / Q000097:blood; D016022:Case-Control Studies; D005260:Female; D006801:Humans; D017827:Machado-Joseph Disease / Q000097:blood*; D008297:Male; D008875:Middle Aged; D009414:Nerve Growth Factors / Q000097:blood*; D010751:Phosphopyruvate Hydratase / Q000097:blood*; D064568:S100 Calcium Binding Protein beta Subunit; D009418:S100 Proteins / Q000097:blood*
chemical_list: D015415:Biomarkers; D009414:Nerve Growth Factors; D064568:S100 Calcium Binding Protein beta Subunit; D009418:S100 Proteins; C579160:S100B protein, human; D010751:Phosphopyruvate Hydratase