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Niemann-Pick Type C (NPC) disease is a rare, genetic, lysosomal disorder with progressive neurodegeneration. Poor understanding of the pathophysiology and lack of blood-based diagnostic markers are major hurdles in the treatment and management of NPC and several additional neurological, lysosomal di...
ORGANISM(S): Mus musculus 
To investigate the putative differential effect of chloride transport on lysosomal ion homeostasis,we generated an ODE mathematical model for this system. Our mathematical model builds upon a previously published model for lysosomal homeostasis (Grabe et al. 2001 J. Gen. Physiol, Ishida et al. 2013 ...
2019-10-22 | MODEL1910220001 | BioModels
A PIKfyve Modulator Combined with an Integrated Stress Response Inhibitor to treat Lysosomal Storage Diseases
Using an siRNA screen we identify a role for GPR65 in the defense against intracellular pathogens. Epithelial cells and macrophages lacking GPR65 exhibited impaired clearance of intracellular bacteria as well as an accumulation of aberrant phagosomes and lysosomes. Transcriptional profiling revealed...
ORGANISM(S): Mus musculus 
Alzheimer’s (AD) and Parkinson’s disease (PD) are neurodegenerative disorders characterized by an accumulation of protein aggregates in the brain. AD is the most common cause of dementia and presents with impairment of memory and cognition. PD results from the loss of dopaminergic neurons which resu...
ORGANISM(S): Homo Sapiens 
Supporting data for "Disruption of lysosomal proteolysis in astrocytes facilitates midbrain proteostasis failure in an early-onset PD model", DOI: T.B.D. Related to Figure 4A and S4A-B. KN43-66 - TMTpro | 4plex | Whole cell proteome - MS3 KN67-72 - TMTpro | 4plex | Enriched Phospho-peptides - MS2 K...
ORGANISM(S): Homo Sapiens (ncbitaxon:9606) 
2022-08-24 | MSV000090202 | MassIVE
Anti-malaria drug chloroquine has been used as an anti-inflammatory agent for treating systemic lupus erythematosus and rheumatoid arthritis, without clear mechanism of action. Here we report that chloroquine potently inhibits the expression of proinflammatory cytokines through transrepression of g...
ORGANISM(S): Homo sapiens 
Lysosomal membrane permeabilization (LMP) or lysosomal membrane damage is commonly associated with aging and age-related diseases. In searching for cellular mechanisms in response to LMP, we used a proteomic approach to identify proteins enriched on damaged lysosomes. This unbiased approach identifi...
ORGANISM(S): Homo sapiens (Human) 
2022-09-07 | PXD028852 | Pride
Lysosomal dysfunction drives a transcriptional and epigenetic signature found in disease-associated microglia in neurodegenerative diseases [RNA-seq]
Lysosomal dysfunction drives a transcriptional and epigenetic signature found in disease-associated microglia in neurodegenerative diseases [ATAC-seq]
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