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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 
The E4 variant of APOE is the primary risk factor for Alzheimer’s Disease. While APOE is primarily expressed by astrocytes, early AD pathology appears predominantly in neurons, including endosomal abnormalities and mitochondrial dysfunction. Lysosomes are poised at the convergence point between thes...
ORGANISM(S): Mus musculus (Mouse) 
2026-01-12 | PXD044942 | Pride
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
Mucolipidosis III gamma (MLIII) is clinically characterized by onset of first symptoms at an average of 5 years such as stiffness of hands and shoulders, claw hand deformities, scoliosis and progressive destruction of hip joints. The disease is caused by mutations in GNPTG encoding the gamma-subunit...
ORGANISM(S): Mus musculus (Mouse) 
2018-06-07 | PXD007547 | Pride
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 
Most lysosomal enzymes require mannose 6-phosphate (M6P) residues for efficient receptor-mediated lysosomal targeting. Although the lack of M6P results in missorting and hypersecretion, selected lysosomal enzymes reach normal levels in lysosomes of various cell types suggesting the existence of M6P-...
ORGANISM(S): Mus musculus (Mouse) 
2015-05-01 | PXD001221 | Pride
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
Lysosomes represent a central degradative compartment of eukaryotes, yet little is known about biogenesis and function of this organelle in parasitic protists. Whereas the mannose-6 phosphate (M6P)-dependent system is dominant for lysosomal targeting in metazoans, oligosaccharide independent sorting...
ORGANISM(S): Trichomonas vaginalis 
2023-07-26 | PXD027545 | Pride
Acute lysosomal membrane damage reduces the cellular population of functional lysosomes. However, these damaged lysosomes have a remarkable recovery potential independent of lysosomal biogenesis and remain unaffected in TFEB/TFE3-depleted cells. We combined proximity labelling based proteomics, bioc...
ORGANISM(S): Homo sapiens (Human) 
2023-03-07 | PXD040191 | Pride
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 
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