Autophagy is disrupted in a knock-in mouse model of juvenile neuronal ceroid lipofuscinosis
about
Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition)Neuronal ceroid lipofuscinosis protein CLN3 interacts with motor proteins and modifies location of late endosomal compartmentsA mammalian autophagosome maturation mechanism mediated by TECPR1 and the Atg12-Atg5 conjugateTau deletion exacerbates the phenotype of Niemann-Pick type C mice and implicates autophagy in pathogenesis.Autophagy in lysosomal storage disordersThe cell biology of disease: lysosomal storage disorders: the cellular impact of lysosomal dysfunctionClarifying lysosomal storage diseasesAutophagy and the ubiquitin-proteasome system: collaborators in neuroprotectionThe pathogenesis of Niemann-Pick type C disease: a role for autophagy?Impairment of homeostasis in lysosomal storage disordersLoss of Cln3 function in the social amoeba Dictyostelium discoideum causes pleiotropic effects that are rescued by human CLN3Distinct early molecular responses to mutations causing vLINCL and JNCL presage ATP synthase subunit C accumulation in cerebellar cellsAutophagy in the Pathogenesis of DiseaseCuring of the [URE3] prion by Btn2p, a Batten disease-related protein.Increased expression of lysosomal acid phosphatase in CLN3-defective cells and mouse brain tissueRetromer Ensures the Degradation of Autophagic Cargo by Maintaining Lysosome Function in DrosophilaATP13A2 regulates mitochondrial bioenergetics through macroautophagyA block of autophagy in lysosomal storage disordersArray-based gene discovery with three unrelated subjects shows SCARB2/LIMP-2 deficiency causes myoclonus epilepsy and glomerulosclerosisCLN3p impacts galactosylceramide transport, raft morphology, and lipid contentMacroautophagy is defective in mucolipin-1-deficient mouse neuronsLysosomal dysfunction and impaired autophagy in a novel mouse model deficient for the lysosomal membrane protein Cln7In Vivo Evidence for Lysosome Depletion and Impaired Autophagic Clearance in Hereditary Spastic Paraplegia Type SPG11Large-scale phenotyping of an accurate genetic mouse model of JNCL identifies novel early pathology outside the central nervous systemLacritin and other autophagy associated proteins in ocular surface health.Lysosomal activity associated with developmental axon pruningPartial correction of the CNS lysosomal storage defect in a mouse model of juvenile neuronal ceroid lipofuscinosis by neonatal CNS administration of an adeno-associated virus serotype rh.10 vector expressing the human CLN3 gene.Validation of microarray data in human lymphoblasts shows a role of the ubiquitin-proteasome system and NF-kB in the pathogenesis of Down syndrome.mTORC1-independent TFEB activation via Akt inhibition promotes cellular clearance in neurodegenerative storage diseasesAlterations in ROS activity and lysosomal pH account for distinct patterns of macroautophagy in LINCL and JNCL fibroblasts.Retinal Degeneration In A Mouse Model Of CLN5 Disease Is Associated With Compromised Autophagy.Novel CLN3 mutation causing autophagic vacuolar myopathy.A novel interaction of CLN3 with nonmuscle myosin-IIB and defects in cell motility of Cln3(-/-) cellsLysosomal fusion and SNARE function are impaired by cholesterol accumulation in lysosomal storage disorders.Disruption of the autophagy-lysosome pathway is involved in neuropathology of the nclf mouse model of neuronal ceroid lipofuscinosisPredicting the fission yeast protein interaction network.The juvenile Batten disease protein, CLN3, and its role in regulating anterograde and retrograde post-Golgi trafficking.A homozygous mutation in KCTD7 links neuronal ceroid lipofuscinosis to the ubiquitin-proteasome system.Reversal of autophagy dysfunction in the TgCRND8 mouse model of Alzheimer's disease ameliorates amyloid pathologies and memory deficitsThe Batten disease gene CLN3 is required for the response to oxidative stress.
P2860
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P2860
Autophagy is disrupted in a knock-in mouse model of juvenile neuronal ceroid lipofuscinosis
description
2006 թուականի Յուլիսին հրատարակուած գիտական յօդուած
@hyw
2006 թվականի հուլիսին հրատարակված գիտական հոդված
@hy
article publié dans la revue scientifique Journal of Biological Chemistry
@fr
artículu científicu espublizáu en 2006
@ast
im Juli 2006 veröffentlichter wissenschaftlicher Artikel
@de
scientific journal article
@en
vedecký článok (publikovaný 2006/07/21)
@sk
vědecký článek publikovaný v roce 2006
@cs
wetenschappelijk artikel (gepubliceerd op 2006/07/21)
@nl
наукова стаття, опублікована в липні 2006
@uk
name
Autophagy is disrupted in a kn ...... neuronal ceroid lipofuscinosis
@ast
Autophagy is disrupted in a kn ...... neuronal ceroid lipofuscinosis
@en
Autophagy is disrupted in a kn ...... neuronal ceroid lipofuscinosis
@nl
type
label
Autophagy is disrupted in a kn ...... neuronal ceroid lipofuscinosis
@ast
Autophagy is disrupted in a kn ...... neuronal ceroid lipofuscinosis
@en
Autophagy is disrupted in a kn ...... neuronal ceroid lipofuscinosis
@nl
prefLabel
Autophagy is disrupted in a kn ...... neuronal ceroid lipofuscinosis
@ast
Autophagy is disrupted in a kn ...... neuronal ceroid lipofuscinosis
@en
Autophagy is disrupted in a kn ...... neuronal ceroid lipofuscinosis
@nl
P2093
P2860
P921
P3181
P356
P1476
Autophagy is disrupted in a kn ...... neuronal ceroid lipofuscinosis
@en
P2093
Ashish C. Massey
Elisa Fossale
Janice A. Espinola
Susan L. Cotman
P2860
P304
20483–20493
P3181
P356
10.1074/JBC.M602180200
P407
P577
2006-07-21T00:00:00Z