Promoting the clearance of neurotoxic proteins in neurodegenerative disorders of ageing
Neurodegenerative disorders of ageing (NDAs) such as Alzheimer disease, Parkinson
disease, frontotemporal dementia, Huntington disease and amyotrophic lateral sclerosis …
disease, frontotemporal dementia, Huntington disease and amyotrophic lateral sclerosis …
[HTML][HTML] Lysosomal acidification dysfunction in microglia: an emerging pathogenic mechanism of neuroinflammation and neurodegeneration
Microglia are the resident innate immune cells in the brain with a major role in orchestrating
immune responses. They also provide a frontline of host defense in the central nervous …
immune responses. They also provide a frontline of host defense in the central nervous …
[HTML][HTML] Rescue of a lysosomal storage disorder caused by Grn loss of function with a brain penetrant progranulin biologic
T Logan, MJ Simon, A Rana, GM Cherf, A Srivastava… - Cell, 2021 - cell.com
GRN mutations cause frontotemporal dementia (GRN-FTD) due to deficiency in progranulin
(PGRN), a lysosomal and secreted protein with unclear function. Here, we found that Grn …
(PGRN), a lysosomal and secreted protein with unclear function. Here, we found that Grn …
[HTML][HTML] Lysosome dysfunction as a cause of neurodegenerative diseases: Lessons from frontotemporal dementia and amyotrophic lateral sclerosis
Frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) are fatal
neurodegenerative disorders that are thought to exist on a clinical and pathological …
neurodegenerative disorders that are thought to exist on a clinical and pathological …
The identity and function of microglia in neurodegeneration
The predominant type of immune cell in the brain is the microglia, a type of tissue-resident
macrophage. In a variety of neurodegenerative settings, microglia alter their transcriptional …
macrophage. In a variety of neurodegenerative settings, microglia alter their transcriptional …
Lysosome trafficking and signaling in health and neurodegenerative diseases
PPY Lie, RA Nixon - Neurobiology of disease, 2019 - Elsevier
Lysosomes, single-membrane organelles defined by a uniquely strong acidic lumenal pH
and high content of acid hydrolases, are the shared degradative compartments of the …
and high content of acid hydrolases, are the shared degradative compartments of the …
Loss of TREM2 rescues hyperactivation of microglia, but not lysosomal deficits and neurotoxicity in models of progranulin deficiency
A Reifschneider, S Robinson, B van Lengerich… - The EMBO …, 2022 - embopress.org
Haploinsufficiency of the progranulin (PGRN)‐encoding gene (GRN) causes frontotemporal
lobar degeneration (GRN‐FTLD) and results in microglial hyperactivation, TREM2 …
lobar degeneration (GRN‐FTLD) and results in microglial hyperactivation, TREM2 …
TDP-43 pathology: from noxious assembly to therapeutic removal
Our understanding of amyotrophic lateral sclerosis and frontotemporal dementia has
advanced dramatically since the discovery of cytoplasmic TAR DNA-binding protein 43 (TDP …
advanced dramatically since the discovery of cytoplasmic TAR DNA-binding protein 43 (TDP …
Lysosomal dysfunction at the centre of Parkinson's disease and frontotemporal dementia/amyotrophic lateral sclerosis
Parkinson's disease (PD) and frontotemporal dementia/amyotrophic lateral sclerosis
(FTD/ALS) are insidious and incurable neurodegenerative diseases that represent a …
(FTD/ALS) are insidious and incurable neurodegenerative diseases that represent a …
Autophagy lysosomal pathway dysfunction in Parkinson's disease; evidence from human genetics
K Senkevich, Z Gan-Or - Parkinsonism & related disorders, 2020 - Elsevier
In recent years, multiple lines of evidence from human genetic and molecular studies have
highlighted the importance of the autophagy lysosomal pathway (ALP) in Parkinson's …
highlighted the importance of the autophagy lysosomal pathway (ALP) in Parkinson's …