Glial nuclear aggregates of superoxide dismutase-1 are regularly present in patients with amyotrophic lateral sclerosis.
Glial nuclear aggregates of superoxide dismutase-1 are regularly present in patients with amyotrophic lateral sclerosis.
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DOI：
10.1007/s00401-011-0805-3
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发表时间：
2011-05
影响因子：
12.7
通讯作者：
Brännström T
中科院分区：
文献类型：
--
作者：
Forsberg K;Andersen PM;Marklund SL;Brännström T
肌萎缩侧索硬化症（ALS）最常见的原因是超氧化物歧化酶-1（SOD 1）的突变。由于有证据表明ALS中涉及非神经元细胞，我们寻找了SOD 1异常的迹象，重点是神经胶质细胞。通过免疫组化检查了9名携带SOD 1突变的ALS患者、51名缺乏此类突变的散发性或家族性ALS患者以及46名对照的脊髓。使用一组对错误折叠的SOD 1种类具有特异性的抗肽抗体。在携带或缺乏SOD 1突变的ALS患者的腹角星形胶质细胞、小胶质细胞和少突胶质细胞的细胞核中，经常检测到颗粒聚集体形式的错误折叠的SOD 1。在神经退行性和非神经系统对照中染色可忽略不计。ALS患者运动神经元胞核内也偶见错误折叠的SOD 1。结果表明，错误折叠的SOD 1存在于神经胶质细胞和运动神经元核通常可能参与ALS的发病机制。本文的在线版本（doi：10.1007/s 00401 -011-0805-3）包含补充材料，可供授权用户使用。
The most common cause of amyotrophic lateral sclerosis (ALS) is mutations in superoxide dismutase-1 (SOD1). Since there is evidence for the involvement of non-neuronal cells in ALS, we searched for signs of SOD1 abnormalities focusing on glia. Spinal cords from nine ALS patients carrying SOD1 mutations, 51 patients with sporadic or familial ALS who lacked such mutations, and 46 controls were examined by immunohistochemistry. A set of anti-peptide antibodies with specificity for misfolded SOD1 species was used. Misfolded SOD1 in the form of granular aggregates was regularly detected in the nuclei of ventral horn astrocytes, microglia, and oligodendrocytes in ALS patients carrying or lacking SOD1 mutations. There was negligible staining in neurodegenerative and non-neurological controls. Misfolded SOD1 appeared occasionally also in nuclei of motoneurons of ALS patients. The results suggest that misfolded SOD1 present in glial and motoneuron nuclei may generally be involved in ALS pathogenesis. The online version of this article (doi:10.1007/s00401-011-0805-3) contains supplementary material, which is available to authorized users.
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影响因子：
14.5
作者：
Jonsson, PA;Graffmo, KS;Marklund, SL
通讯作者：
Marklund, SL
影响因子：
4.8
作者：
Ge, WW;Wen, WY;Strong, MJ
通讯作者：
Strong, MJ
DOI：
10.1097/01.jnen.0000248545.36046.3c
发表时间：
2006-12-01
影响因子：
3.2
作者：
Jonsson, P. Andreas;Graffmo, Karin S.;Marklund, Stefan L.
通讯作者：
Marklund, Stefan L.
影响因子：
12.7
作者：
Kerman, Aaron;Liu, Hsueh-Ning;Chakrabartty, Avijit
通讯作者：
Chakrabartty, Avijit
影响因子：
4.7
作者：
Bergemalm, Daniel;Forsberg, Karin;Marklund, Stefan L.
通讯作者：
Marklund, Stefan L.