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ProteomicsMFSD1 KO Liver - Lysosomes are a major site of intracellular acidic hydrolase-mediated proteolysis and cellular degradation
Lysosomes are a major site of intracellular acidic hydrolase-mediated proteolysis and cellular degradation. The export of low-molecular catabolic end products from the lysosomal lumen to the cytosol is facilitated by polytopic transmembrane proteins which mediate secondary active or passive transpor...
ORGANISM(S): Mus musculus (Mouse) 
2019-10-28 | PXD014241 | Pride
Lysosome Liver Mfsd1 
ProteomicsCLN6 deficiency causes selective changes in the lysosomal protein composition
Neuronal Ceroid Lipofuscinosis 6 (NCL6) is a neurodegenerative, lysosomal storage disease. It is caused by a deficiency of the transmembrane protein ceroid-lipofuscinosis neuronal protein 6 (CLN6) that resides in the endoplasmic reticulum. In this project the lysosomal proteome changes in NCL6 were ...
ORGANISM(S): Mus musculus (Mouse) 
2024-10-28 | PXD016599 | Pride
Cln6 Lysosome Nclf 
ProteomicsProteome Composition of Lysosomes and Autophagosomes after Proteasome Inhibition
Lysosomes are a major site of intracellular acidic hydrolase-mediated proteolysis and cellular degradation in a membrane-enclosed organelle. Alternatively, soluble, ubiquitin-bound proteins are degraded via the proteasome, a megadalton protein complex within the cytoplasm. The interplay between both...
ORGANISM(S): Homo sapiens (Human) 
2020-04-20 | PXD015243 | Pride
Proteaphagy Lysosome Proteasome 
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 
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 
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