Pathobiology of the autophagy-lysosomal pathway in the Huntington's disease brain.

ABSTRACT:

Background

Accumulated levels of mutant huntingtin protein (mHTT) and its fragments are considered contributors to the pathogenesis of Huntington's disease (HD). Stimulating autophagy may enhance clearance of mHTT and its aggregates which has been considered as a possible therapeutic strategy. However, the role and competence of the autophagy-lysosomal pathway (ALP) during HD progression in the human disease remains largely unknown.

Methods

Here, we used multiplex confocal and ultrastructural immunocytochemical analyses of ALP functional markers in relation to mHTT aggresome pathology in striatum and the less affected cortex or cerebellum of HD brains staged from Grade HD2 to HD4 by Vonsattel neuropathological criteria compared to controls.

Results

Immunolabeling reve

SUBMITTER: Berg MJ 

PROVIDER: S-EPMC12598841 | biostudies-literature | 2025 Nov

REPOSITORIES: biostudies-literature