The pathological TDP-43 protein expression in the central nervous system of motor neuron disease
10.3760/cma.j.issn.0578-1426.2015.01.009
- VernacularTitle:运动神经元病与TDP-43蛋白的病理性表达
- Author:
Mingwei ZHU
;
Jia LIU
;
Luning WANG
;
Qiuping GUI
- Publication Type:Journal Article
- Keywords:
Motor neuron disease;
TDP-43;
Ubiquitin;
Inclusion;
Brain;
Spinal cord
- From:
Chinese Journal of Internal Medicine
2015;54(1):31-34
- CountryChina
- Language:Chinese
-
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.Conclusion 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.
