Departamento de Bioquímica y Biología Molecular, Facultad de Medicina, Instituto Maimónides de Investigación Biomédica de Córdoba (IMIBIC)/Universidad de Córdoba, Córdoba, Spain.
J Neurol Sci. 2012 Apr 15;315(1-2):133-6. doi: 10.1016/j.jns.2011.12.014. Epub 2012 Jan 16.
Huntington's disease (HD) is a neurodegenerative genetic disorder caused by expansion of polyglutamine repeats in the huntingtin gene and characterised by the loss of striatal and cortical neurons. Few studies to date have focussed on peripheral neurotrophic-factor levels in patients with HD.
To measure plasma NGF levels in Huntington's disease and investigate their correlation with disease intensity.
Nineteen patients with HD and nineteen age- and sex-matched healthy subjects took part in this cross-sectional study. Plasma levels of NGF, BDNF, GDNF, nitrotyrosine, and myeloperoxidase (MPO) were measured; lactate dehydrogenase (LDH) levels were determined and white blood cell (WBC) counts were evaluated.
NGF levels were significantly lower, nitrotyrosine levels were higher and LDH activity was greater in HD patients than in healthy subjects. There was no significant difference in MPO levels or WBC counts, whereas the MPO/WBC ratio was considerably higher in HD patients. The data obtained suggested that biochemical and haematological changes correlated with disease severity.
NGF levels are lower in HD patients than in healthy subjects. However, further research is required to confirm the role of NGF in HD.
亨廷顿病(HD)是一种由亨廷顿基因中多聚谷氨酰胺重复扩展引起的神经退行性遗传疾病,其特征是纹状体和皮质神经元的丧失。迄今为止,很少有研究关注亨廷顿病患者外周神经营养因子水平。
测量亨廷顿病患者的血浆 NGF 水平,并探讨其与疾病严重程度的相关性。
本横断面研究纳入了 19 名亨廷顿病患者和 19 名年龄和性别匹配的健康对照者。测量了血浆 NGF、BDNF、GDNF、硝基酪氨酸和髓过氧化物酶(MPO)水平;测定了乳酸脱氢酶(LDH)水平,并评估了白细胞(WBC)计数。
与健康对照组相比,亨廷顿病患者的 NGF 水平显著降低,硝基酪氨酸水平升高,LDH 活性更高。MPO 水平或 WBC 计数无显著差异,而 MPO/WBC 比值在亨廷顿病患者中显著升高。数据表明生化和血液学变化与疾病严重程度相关。
亨廷顿病患者的 NGF 水平低于健康对照组。然而,需要进一步研究来证实 NGF 在亨廷顿病中的作用。