Lysosomal dysfunction at the centre of Parkinson's disease and frontotemporal dementia/amyotrophic lateral sclerosis

RL Wallings, SW Humble, ME Ward… - Trends in …, 2019 - cell.com
Parkinson's disease (PD) and frontotemporal dementia/amyotrophic lateral sclerosis
(FTD/ALS) are insidious and incurable neurodegenerative diseases that represent a …

Cathepsin D—managing the delicate balance

O Mijanovic, AI Petushkova, A Brankovic, B Turk… - Pharmaceutics, 2021 - mdpi.com
Lysosomal proteases play a crucial role in maintaining cell homeostasis. Human cathepsin
D manages protein turnover degrading misfolded and aggregated proteins and favors …

The subcellular arrangement of alpha-synuclein proteoforms in the Parkinson's disease brain as revealed by multicolor STED microscopy

TE Moors, CA Maat, D Niedieker, D Mona… - Acta …, 2021 - Springer
Various post-translationally modified (PTM) proteoforms of alpha-synuclein (aSyn)—
including C-terminally truncated (CTT) and Serine 129 phosphorylated (Ser129-p) aSyn …

Sequential stages and distribution patterns of aging-related tau astrogliopathy (ARTAG) in the human brain

GG Kovacs, SX Xie, JL Robinson, EB Lee… - Acta neuropathologica …, 2018 - Springer
Aging-related tau astrogliopathy (ARTAG) describes tau pathology in astrocytes in different
locations and anatomical regions. In the present study we addressed the question of …

Distinct ultrastructural phenotypes of glial and neuronal alpha-synuclein inclusions in multiple system atrophy

C Böing, M Di Fabrizio, D Burger, JGJM Bol… - Brain, 2024 - academic.oup.com
Abstract Multiple System Atrophy is characterized pathologically by the accumulation of
alpha-synuclein (aSyn) into glial cytoplasmic inclusions (GCIs). The mechanism underlying …

Autophagy mediates the clearance of oligodendroglial SNCA/alpha-synuclein and TPPP/p25A in multiple system atrophy models

P Mavroeidi, F Arvanitaki, M Vetsi, S Becker… - Autophagy, 2022 - Taylor & Francis
Accumulation of the neuronal protein SNCA/alpha-synuclein and of the oligodendroglial
phosphoprotein TPPP/p25A within the glial cytoplasmic inclusions (GCIs) represents the key …

Neuropathology and pathogenesis of extrapyramidal movement disorders: a critical update—I. Hypokinetic-rigid movement disorders

KA Jellinger - Journal of Neural Transmission, 2019 - Springer
Extrapyramidal movement disorders include hypokinetic rigid and hyperkinetic or mixed
forms, most of them originating from dysfunction of the basal ganglia (BG) and their …

A historical review of multiple system atrophy with a critical appraisal of cellular and animal models

DJ Marmion, W Peelaerts, JH Kordower - Journal of Neural Transmission, 2021 - Springer
Multiple system atrophy (MSA) is a progressive neurodegenerative disorder characterized
by striatonigral degeneration (SND), olivopontocerebellar atrophy (OPCA), and …

Targeting of lysosomal pathway genes for Parkinson's disease modification: insights from cellular and animal models

T Abe, T Kuwahara - Frontiers in Neurology, 2021 - frontiersin.org
Previous genetic studies on hereditary Parkinson's disease (PD) have identified a set of
pathogenic gene mutations that have strong impacts on the pathogenicity of PD. In addition …

Reducing INS-IGF1 signaling protects against non-cell autonomous vesicle rupture caused by SNCA spreading

CA Sandhof, SO Hoppe, S Druffel-Augustin… - Autophagy, 2020 - Taylor & Francis
Aging is associated with a gradual decline of cellular proteostasis, giving rise to devastating
protein misfolding diseases, such as Alzheimer disease (AD) or Parkinson disease (PD) …