title: [The pathological TDP-43 protein expression in the central nervous system of motor neuron disease].
abstract: OBJECTIVE
  To understand pathological TDP-43 features in the central nervous systems of patients with clinically and autopsy confirmed motor neuron disease (MND).
  
  
  METHODS
  The clinical and histopathological features of 4 cases with MND confirmed by autopsy were summarized; anti-ubiquitin (Ub) and anti-TDP-43 immunohistochemical staining were carried out on tissue of brains and spinal cords from 4 cases with MND and 3 control cases without history of neurological disorders.
  
  
  RESULTS
  These 4 cases presented with typical clinical and histologic features of MND. Ub-positive inclusions were observed in brain and spinal cord from 3 cases with the Ub-positive inclusions of skein- round- and lewy body- like structures. Strong TDP-43 pathological staining in brain and spinal cord was identified in 2 cases with MND presented as neuronal and glial cytoplasmic inclusions with various shapes. The TDP-43 positive inclusions were widely distributed in the motor cortex of brain and the anterior horn of spinal cord. TDP-43 weak staining in the spinal cord tissue was observed in 1 case with MND. No Ub- and TDP-43 positive inclusions were found in 3 control cases.
  
  
  CONCLUSIONS
  There is widespread pathological TDP-43 expression in the central nervous system of MND. TDP-43 positive inclusions in MND have relatively high specificity. It is worth further study on their formation mechanism.
authors: Mingwei
authors: M
authors: Zhu
authors: Jia
authors: J
authors: Liu
authors: Department of Geriatric Neurology, Chinese PLA General Hospital, Beijing 100853, China. Email: LNW_301@163.com.
authors: Luning
authors: L
authors: Wang
authors: Qiuping
authors: Q
authors: Gui
mesh_terms: D001921:Brain / Q000378:metabolism*; D016022:Case-Control Studies; D002540:Cerebral Cortex / Q000378:metabolism; D004268:DNA-Binding Proteins / Q000378:metabolism*; D006801:Humans; D007150:Immunohistochemistry; D002479:Inclusion Bodies; D016472:Motor Neuron Disease / Q000473:pathology*; D025801:Ubiquitin / Q000032:analysis*
chemical_list: D004268:DNA-Binding Proteins; D025801:Ubiquitin