Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs) - Test2 - elhamkhani - TriplyDB

Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs)

Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs)

http://www.wikidata.org/entity/Q24291928

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Defective lysosomal arginine transport in juvenile Batten disease A galactosylceramide binding domain is involved in trafficking of CLN3 from Golgi to rafts via recycling endosomes Neuronal ceroid lipofuscinoses are connected at molecular level: interaction of CLN5 protein with CLN2 and CLN3 Neuronal ceroid lipofuscinosis protein CLN3 interacts with motor proteins and modifies location of late endosomal compartments Kufs disease, the major adult form of neuronal ceroid lipofuscinosis, caused by mutations in CLN6 Membrane trafficking and mitochondrial abnormalities precede subunit c deposition in a cerebellar cell model of juvenile neuronal ceroid lipofuscinosis FRET-assisted determination of CLN3 membrane topology Loss of Cln3 function in the social amoeba Dictyostelium discoideum causes pleiotropic effects that are rescued by human CLN3 Neurodegeneration and Epilepsy in a Zebrafish Model of CLN3 Disease (Batten Disease)Distinct early molecular responses to mutations causing vLINCL and JNCL presage ATP synthase subunit C accumulation in cerebellar cells Lysosome and calcium dysregulation in Alzheimer's disease: partners in crime Increased expression of lysosomal acid phosphatase in CLN3-defective cells and mouse brain tissue Lysosomal Re-acidification Prevents Lysosphingolipid-Induced Lysosomal Impairment and Cellular Toxicity Autophagy is disrupted in a knock-in mouse model of juvenile neuronal ceroid lipofuscinosis Alterations in ROS activity and lysosomal pH account for distinct patterns of macroautophagy in LINCL and JNCL fibroblasts.A novel interaction of CLN3 with nonmuscle myosin-IIB and defects in cell motility of Cln3(-/-) cells Lysosomal fusion and SNARE function are impaired by cholesterol accumulation in lysosomal storage disorders.The juvenile Batten disease protein, CLN3, and its role in regulating anterograde and retrograde post-Golgi trafficking.Neurodevelopmental delay in the Cln3Deltaex7/8 mouse model for Batten disease.The neuronal ceroid-lipofuscinoses.The intracellular location and function of proteins of neuronal ceroid lipofuscinoses.Unbiased Cell-based Screening in a Neuronal Cell Model of Batten Disease Highlights an Interaction between Ca2+ Homeostasis, Autophagy, and CLN3 Protein Function.Investigating the interaction of saposin C with POPS and POPC phospholipids: a solid-state NMR spectroscopic study.Protecting cells by protecting their vulnerable lysosomes: Identification of a new mechanism for preserving lysosomal functional integrity upon oxidative stress.Photoreceptor phagosome processing defects and disturbed autophagy in retinal pigment epithelium of Cln3Δex1-6 mice modelling juvenile neuronal ceroid lipofuscinosis (Batten disease).Thematic review series: lipid posttranslational modifications. Lysosomal metabolism of lipid-modified proteins.Membrane traffic and turnover in TRP-ML1-deficient cells: a revised model for mucolipidosis type IV pathogenesis Impaired proteolysis underlies autophagic dysfunction in Niemann-Pick type C disease Autophagy failure in Alzheimer's disease and the role of defective lysosomal acidification Antigen presenting cell abnormalities in the Cln3(-/-) mouse model of juvenile neuronal ceroid lipofuscinosis WASH is required for lysosomal recycling and efficient autophagic and phagocytic digestion.Analysis of NCL Proteins from an Evolutionary Standpoint.Misrouting of v-ATPase subunit V0a1 dysregulates lysosomal acidification in a neurodegenerative lysosomal storage disease model.Anilinomethylrhodamines: pH sensitive probes with tunable photophysical properties by substituent effect.Update of the mutation spectrum and clinical correlations of over 360 mutations in eight genes that underlie the neuronal ceroid lipofuscinoses.btn1 affects endocytosis, polarization of sterol-rich membrane domains and polarized growth in Schizosaccharomyces pombe.AP-1 and AP-3 facilitate lysosomal targeting of Batten disease protein CLN3 via its dileucine motif.Two motifs target Batten disease protein CLN3 to lysosomes in transfected nonneuronal and neuronal cells.High lumenal chloride in the lysosome is critical for lysosome function.Live-cell Microscopy and Fluorescence-based Measurement of Luminal pH in Intracellular Organelles.

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Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs)

http://www.wikidata.org/entity/Q24291928

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2001 nî lūn-bûn

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2001 թուականի Նոյեմբերին հրատարակուած գիտական յօդուած

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2001 թվականի նոյեմբերին հրատարակված գիտական հոդված

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2001年の論文

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name

Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs)

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Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs)

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Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs)

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Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs)

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Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs)

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Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs)

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Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs)

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Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs)

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Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs)

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Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs)

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Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs)

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Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs)

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J.0014-2956.2001.02530.X

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Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs)

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I Järvelä

http://www.w3.org/2001/XMLSchema#string

J M Holopainen

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J Saarikoski

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P K Kinnunen

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P2860

Classical late infantile neuronal ceroid lipofuscinosis fibroblasts are deficient in lysosomal tripeptidyl peptidase I

Defective intracellular transport of CLN3 is the molecular basis of Batten disease (JNCL)

The neuronal ceroid lipofuscinoses in human EPMR and mnd mutant mice are associated with mutations in CLN8

The neuronal ceroid lipofuscinosis CLN8 membrane protein is a resident of the endoplasmic reticulum

Biosynthesis and intracellular targeting of the CLN3 protein defective in Batten disease

Association of mutations in a lysosomal protein with classical late-infantile neuronal ceroid lipofuscinosis

CLN5, a novel gene encoding a putative transmembrane protein mutated in Finnish variant late infantile neuronal ceroid lipofuscinosis

Mutations in the palmitoyl protein thioesterase gene causing infantile neuronal ceroid lipofuscinosis

Rapid diagnostic test for the major mutation underlying Batten disease

The subcellular location of the yeast Saccharomyces cerevisiae homologue of the protein defective in the juvenile form of Batten disease.

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lysosomal lumen acidification

CLN3 lysosomal/endosomal transmembrane protein, battenin

CLN5 intracellular trafficking protein

CLN6 transmembrane ER protein

Palmitoyl-protein thioesterase 1