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ProteomicsLC-MS/MS analysis of soluble lysosomal enzymes
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
Mucolipidosis Lysosomal proteomics 
ProteomicsTargeting of non-phosphorylated lysosomal enzymes involves secretion and Lrp1-mediated recapture
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
Low density lipoprotein-related receptor/ lysosomal targeting/ mannose 6-phosphate/ receptor-mediated endocytosis/ sortilin 
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
ProteomicsProteomic analysis of Trichomonas vaginalis phagolysosome, lysosomal targeting, and unconventional secretion of cysteine peptidases
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
Trichomonas vaginalis Glycosylation Mannose-6-phosphate receptor Phagolysosome Cysteine peptidase Proteome 
ProteomicsA Universal GlycoDesign for Lysosomal Replacement Enzymes to Improve Circulation Time and Biodistribution
Currently available enzyme replacement therapies for lysosomal storage diseases are limited in their effectiveness due in part to short circulation times and suboptimal biodistribution of the therapeutic enzymes. We previously engineered Chinese hamster ovary (CHO) cells to produce -galactosidase A ...
ORGANISM(S): Homo sapiens (Human) 
2023-02-13 | PXD038783 | Pride
Glycoproteomics Lc-ms Therapeutics Orbitrap 
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
ProteomicsThe Glycosylation Design Space for Recombinant Lysosomal Replacement Enzymes Produced in CHO Cells
Lysosomal storage diseases are rare life-threatening disorders caused by deficiency of a lysosomal enzyme, and delivery of a recombinant replacement enzyme is the primary therapy for several of these diseases. The structures of N-glycans on recombinant replacement enzymes are important for their the...
ORGANISM(S): Homo sapiens (Human) Cricetulus griseus (Chinese hamster) (Cricetulus barabensis griseus) 
2019-05-31 | PXD013140 | Pride
N-glycoproteomics Enzyme replacement therapy Glycoengineering 
TranscriptomicsComparison of bone marrow-derived macrophages from BALB/c and C57BL/6 mice with and without interferon-gamma stimulation.
ABSTRACT Macrophages are essential components of the innate immune system and crucial for pathogen elimination in early stages of infection. We previously observed that bone marrow-derived macrophages (BMM) from C57BL/6 mice exhibited increased killing activity against Burkholderia pseudomallei comp...
ORGANISM(S): Mus musculus 
2014-04-15 | E-GEOD-39349 | biostudies-arrayexpress
GenomicsLysosomal multi-omics reveals altered sphingolipid catabolism as driver of lysosomal dysfunction in the aging brain.
Lysosomal multi-omics reveals altered sphingolipid catabolism as driver of lysosomal dysfunction in the aging brain.
PRJNA1363158 | ENA
UnknownMannose 6 phosphorylation of lysosomal enzymes controls B cell functions.
Not available
S-EPMC4298682 | biostudies-literature
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