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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 
UnknownPrevention of Photoreceptor Cell Loss in a Cln6nclf Mouse Model of Batten Disease Requires CLN6 Gene Transfer to Bipolar Cells.
Not available
S-EPMC5993939 | biostudies-literature
UnknownCLN6-related continuum phenotype caused by aberrant splicing.
Not available
S-EPMC11803302 | biostudies-literature
UnknownA CLN6-CLN8 complex recruits lysosomal enzymes at the ER for Golgi transfer.
Not available
S-EPMC7410054 | biostudies-literature
UnknownAltered biometal homeostasis is associated with CLN6 mRNA loss in mouse neuronal ceroid lipofuscinosis.
Not available
S-EPMC3683166 | biostudies-literature
UnknownGene Therapy Corrects Brain and Behavioral Pathologies in CLN6-Batten Disease.
Not available
S-EPMC6822284 | biostudies-literature
UnknownNeuronal progenitor cells-based metabolomics study reveals dysregulated lipid metabolism and identifies putative biomarkers for CLN6 disease.
Not available
S-EPMC10613621 | biostudies-literature
UnknownCln6 mutants associated with neuronal ceroid lipofuscinosis are degraded in a proteasome-dependent manner.
Not available
S-EPMC2674128 | biostudies-literature
UnknownKufs disease, the major adult form of neuronal ceroid lipofuscinosis, caused by mutations in CLN6.
Not available
S-EPMC3146726 | biostudies-literature
UnknownElectroretinography data from ovine models of CLN5 and CLN6 neuronal ceroid lipofuscinoses.
Not available
S-EPMC8187955 | biostudies-literature
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