Abstract | OBJECTIVE: METHODS: RESULTS: Immunohistochemical study showed that the three proteins were mainly expressed in the dysmorphic neurons and balloon cells/giant cells of TSC and FCD IIb. The positivity was more intense in the dysmorphic neurons than the other cell types. Immunostaining for Beclin-1 showed granular or diffuse cytoplasmic positivity, in addition to the strong expression in axons. On the other hand, LC3 showed diffuse or perinuclear cytoplasmic expression. The staining for p62 was mainly cytoplasmic or perinuclear and sometimes nuclear. In FCD type I, only individual cells showed positive expression for the three proteins. The number of Beclin-1 and LC3-positive cells was larger in TSC group, followed by FCD IIb group and FCD I group.And there were significant differences between TSC group and FCD I group, as well as FCD IIb group and FCD I group (P<0.05). Quantitative expression of LC3 protein by Western blot showed smaller amount in TSC group, followed by FCD IIb group and FCD I group. CONCLUSIONS: The dysmorphic neurons and balloon cells/giant cells of TSC and FCD IIb show abnormality in autophagy, resulting in intracytoplasmic protein accumulation. There are differences in molecular pathogenesis in these cell types.
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Authors | Shiyun Chen, Yueshan Piao, Yongjuan Fu, Zhuo Li, Cuicui Liu, Dehong Lu |
Journal | Zhonghua bing li xue za zhi = Chinese journal of pathology
(Zhonghua Bing Li Xue Za Zhi)
Vol. 44
Issue 5
Pg. 305-9
(May 2015)
ISSN: 0529-5807 [Print] China |
PMID | 26178210
(Publication Type: Journal Article)
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Chemical References |
- Autophagy-Related Proteins
- BECN1 protein, human
- Beclin-1
- MAP1LC3A protein, human
- Microtubule-Associated Proteins
- P62 protein, human
- RNA-Binding Proteins
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Topics |
- Autophagy
- Autophagy-Related Proteins
(metabolism)
- Beclin-1
(metabolism)
- Blotting, Western
- Brain
(metabolism)
- Cytoplasm
(metabolism)
- Epilepsy
(metabolism)
- Female
- Giant Cells
(metabolism)
- Humans
- Immunohistochemistry
- Male
- Malformations of Cortical Development
(metabolism)
- Malformations of Cortical Development, Group I
(metabolism)
- Microtubule-Associated Proteins
(metabolism)
- Neurons
(metabolism)
- RNA-Binding Proteins
(metabolism)
- Tuberous Sclerosis
(metabolism)
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