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Abstract Expansion of a polyglutamine tract within ataxin -1 causes spinocerebellar ataxia type 1 (SCA1). In this study, we used the yeast two-hybrid system to identify an ataxin -1-interacting protein ...
) and the mutant ataxin - 3 aggregates in spinocerebellar ataxia type 3 /Machado-Joseph disease (SCA3/MJD) (9). Two protein quality control systems, the Ubiquitin Proteasome System (UPS) and Autophagy Lysosome Pathway ...
). The nucleation complex is also composed by Beclin 1 ( BCL - 2 -interacting protein ). Beclin-1 is negatively regulated through the interaction with BCL - 2 and BCL -XL at the ER membrane (48). Vesicle expansion is carried ...
), subcortical WM immediate adjacent to U-fiber (ROI- 2 ), WM with extensive degeneration (ROI- 3 ), WM with no or mild degeneration (ROI-4), cerebral cortex above ROI- 3 (ROI-5), and cerebral cortex above ROI-4 (ROI-6 ...
) GABAergic deficiency is a potential factor for refractory epilepsy associated with FCD; ( 2 ) the “broad pattern” may indicate epileptic vulnerability located outside the dysplastic area; and ( 3 ) KCC2 ...
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