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MetabolomicsNPC1 regulates the distribution of phosphatidylinositol 4 kinases at Golgi and Lysosome membranes
Cholesterol and phosphoinositides (PI) are two critically important lipids that are found in cellular membranes and dysregulated in many disorders. Therefore, uncovering molecular pathways connecting these essential lipids may offer new therapeutic insights. We report that loss of function of lysoso...
2021-09-15 | MTBLS2612 | MetaboLights
MetabolomicsLysosome Lipid Signaling from the Periphery to Neurons Regulates Longevity

Lysosomes are key cellular organelles that metabolize extra- and intra-cellular substrates. Alterations in lysosomal metabolism are implicated in aging-associated metabolic and neurodegenerative diseases. However, how lysosomal metabolism actively coordinates the metabolic and nervous systems to ...

2022-05-06 | MTBLS4654 | MetaboLights
MetabolomicsELAPOR1 induces the classical/progenitor subtype and contributes to reduced disease aggressiveness through metabolic reprogramming in pancreatic cancer
Pancreatic ductal adenocarcinoma (PDAC) is a heterogeneous disease with distinct molecular subtypes described as classical/progenitor and basal-like/squamous PDAC. We hypothesized that integrative transcriptome and metabolome approaches can identify candidate genes whose inactivation contributes to ...
2024-04-02 | MTBLS9418 | MetaboLights
ProteomicsProteomic study in Pompe disease
The raw data of SWATH proteomic analysis of plasma in Pompe disease patients and controls
ORGANISM(S): Homo Sapiens (ncbitaxon:9606) 
MSV000086114 | MassIVE
TranscriptomicsExpression data from brain, liver and spleen of Npc1-/- mice
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 
2012-12-27 | E-GEOD-39621 | biostudies-arrayexpress
ProteomicsLysosomal storage disease proteo/lipidomic profiling using nMOST links ferritinophagy with mitochondrial iron deficiencies in cells lacking NPC2
Lysosomal storage diseases (LSDs) comprised ~50 gene loci causing the accumulation of cellular material in the lysosome and associated defects in lysosomal function, but systematic molecular phenotyping is lacking. Here, we apply a nanoflow-based multi-omic single-shot technology (nMOST) workflow to...
ORGANISM(S): Homo sapiens (Human) 
2024-10-08 | PXD049336 | Pride
Ferritinophagy Multiplexed tmt proteomics Oxphos Lysosomal storage disorders Mitochondrial function 
Proteomicslysosome proteomics in Niemann-Pick Type C disease model cells (CHOwt and NPC1-null cells)
Increasing evidence implicates lysosomal dysfunction in the pathogenesis of neurodegenerative diseases, including the rare inherited lysosomal storage disorders (LSDs) and the most common neurodegenerative diseases, such as Alzheimer’s and Parkinson’s disease (AD and PD). The goal of this work was t...
ORGANISM(S): Homo sapiens (Human) 
2018-03-21 | PXD008438 | Pride
Magnetic nanoparticles lysosome enrichment Npc1-null cells Chowt cells Lysosome Lc-ms/ms 
UnknownLysosome biogenesis in health and disease.
Not available
S-EPMC6368902 | biostudies-literature
TranscriptomicsLCM-based microarray analysis of neuronal vulnerability in the mouse model of Sanfilippo syndrome (MPSIIIB)
Sanfilippo syndrome type B (MPS III B) is an autosomal recessive, neurodegenerative disease of children, characterized by profound mental retardation and dementia. The primary cause is mutation in the NAGLU gene, resulting in deficiency of N-acetylglucosaminidase and lysosomal accumulation of hepara...
ORGANISM(S): Mus musculus 
2009-04-22 | E-GEOD-15758 | biostudies-arrayexpress
ProteomicsIdentification of lysosomal phospholipases in context of Batten disease Cln3
Batten disease, the most prevalent form of neurodegeneration in children, is caused by mutations in the CLN3 gene, which encodes a lysosomal transmembrane protein. The loss of CLN3 leads to significant accumulation of glycerophosphodiesters (GPDs), the end products of glycerophospholipid catabolism ...
ORGANISM(S): Homo Sapiens (ncbitaxon:9606) 
2023-08-23 | MSV000092731 | MassIVE
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