Ultrastructural diversity of inclusions and aggregations in the lumbar spinal cord of SOD1-G93A transgenic mice

被引:17
作者
Guo, Yansu
Li, Chunyan [1 ]
Wu, Dongxia
Wu, Shuyu
Yang, Cheng
Liu, Yang
Wu, Hongran
Li, Zhongyao
机构
[1] Hebei Med Univ, Hosp 2, Dept Neurol, Shijiazhuang 050000, Hebei, Peoples R China
基金
中国国家自然科学基金;
关键词
Amyotrophic lateral sclerosis; Transgenic mouse; Spinal cord; Ultrastructure; Inclusion; AMYOTROPHIC-LATERAL-SCLEROSIS; CU; ZN SUPEROXIDE-DISMUTASE; ALS-LINKED SOD1; ASTROCYTIC HYALINE INCLUSIONS; MOTOR-NEURON DEATH; SUPEROXIDE-DISMUTASE-1; SOD1; GOLGI-APPARATUS; GENE MUTATION; MOUSE MODEL; NEUROPATHOLOGY;
D O I
10.1016/j.brainres.2010.07.025
中图分类号
Q189 [神经科学];
学科分类号
071006 ;
摘要
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by selective motor neuron death. We report the characteristics of ultrastructural pathological changes of inclusions and aggregations in the neuronal axons, glial cells and ventral roots of lumbar spinal cord in SOD1-G93A transgenic mice using light and electron transmission microscope at different stages of disease. The most noteworthy is that mutant SOD1 accumulations in the cytoplasm of motor neurons precede the numerous inclusions. Inclusions manifested differently according to the specified locations. This study provided further information to the previous reports about pathological changes of ALS. (C) 2010 Elsevier B.V. All rights reserved.
引用
收藏
页码:234 / 244
页数:11
相关论文
共 50 条
  • [41] Assessing the therapeutic impact of resveratrol in ALS SOD1-G93A mice with electrical impedance myography
    Nagy, Janice A. A.
    Semple, Carson
    Lo, PuiChi
    Rutkove, Seward B. B.
    FRONTIERS IN NEUROLOGY, 2022, 13
  • [42] Muscle Expression of SOD1G93A Modulates microRNA and mRNA Transcription Pattern Associated with the Myelination Process in the Spinal Cord of Transgenic Mice
    Dobrowolny, Gabriella
    Bernardini, Camilla
    Martini, Martina
    Baranzini, Mirko
    Barba, Marta
    Musaro, Antonio
    FRONTIERS IN CELLULAR NEUROSCIENCE, 2015, 9 : 1 - 12
  • [43] Therapeutic Potential of N-Acetyl-Glucagon-Like Peptide-1 in Primary Motor Neuron Cultures Derived From Non-Transgenic and SOD1-G93A ALS Mice
    Sun, Hui
    Knippenberg, Sarah
    Thau, Nadine
    Ragancokova, Daniela
    Koerner, Sonja
    Huang, Dongya
    Dengler, Reinhard
    Doehler, Klaus
    Petri, Susanne
    CELLULAR AND MOLECULAR NEUROBIOLOGY, 2013, 33 (03) : 347 - 357
  • [44] An oligomer-specific antibody improved motor function and attenuated neuropathology in the SOD1-G93A transgenic mouse model of ALS
    Dong, Quan-xiu
    Zhu, Jie
    Liu, Shu-ying
    Yu, Xiao-lin
    Liu, Rui-tian
    INTERNATIONAL IMMUNOPHARMACOLOGY, 2018, 65 : 413 - 421
  • [45] Galectin-1 deficiency improves axonal swelling of motor neurones in SOD1G93A transgenic mice
    Kobayakawa, Y.
    Sakumi, K.
    Kajitani, K.
    Kadoya, T.
    Horie, H.
    Kira, J-i
    Nakabeppu, Y.
    NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY, 2015, 41 (02) : 227 - 244
  • [46] Repurposing carbamazepine for the treatment of amyotrophic lateral sclerosis in SOD1-G93A mouse model
    Zhang, Jing-Jing
    Zhou, Qin-Ming
    Chen, Sheng
    Le, Wei-Dong
    CNS NEUROSCIENCE & THERAPEUTICS, 2018, 24 (12) : 1163 - 1174
  • [47] Oxidative Stress and Autophagic Alteration in Brainstem of SOD1-G93A Mouse Model of ALS
    An, Ting
    Shi, Pengxiao
    Duan, Weisong
    Zhang, Shipan
    Yuan, Pin
    Li, Zhongyao
    Wu, Dongxia
    Xu, Zuoshang
    Li, Chunyan
    Guo, Yansu
    MOLECULAR NEUROBIOLOGY, 2014, 49 (03) : 1435 - 1448
  • [48] Early Excitability Changes in Lumbar Motoneurons of Transgenic SOD1G85R and SOD1G93A-Low Mice
    Pambo-Pambo, Arnaud
    Durand, Jacques
    Gueritaud, Jean-Patrick
    JOURNAL OF NEUROPHYSIOLOGY, 2009, 102 (06) : 3627 - 3642
  • [49] Monoaminergic control of spinal locomotor networks in SOD1G93A newborn mice
    Milan, Lea
    Barriere, Gregory
    De Deurwaerdere, Philippe
    Cazalets, Jean-Rene
    Bertrand, Sandrine S.
    FRONTIERS IN NEURAL CIRCUITS, 2014, 8
  • [50] Dose-dependent efficacy of ALS-human mesenchymal stem cells transplantation into cisterna magna in SOD1-G93A ALS mice
    Kim, Heejaung
    Kim, Hyun Young
    Choi, Mi Ran
    Hwang, Sejin
    Nam, Ki-Hoan
    Kim, Hyoung-Chin
    Han, Jin Soo
    Kim, Kyung Suk
    Yoon, Hyun Soo
    Kim, Seung H.
    NEUROSCIENCE LETTERS, 2010, 468 (03) : 190 - 194