Disruption of the autophagy-lysosome pathway is involved in neuropathology of the nclf mouse model of neuronal ceroid lipofuscinosis - Wikidata - wikimedia - TriplyDB

Disruption of the autophagy-lysosome pathway is involved in neuropathology of the nclf mouse model of neuronal ceroid lipofuscinosis

Disruption of the autophagy-lysosome pathway is involved in neuropathology of the nclf mouse model of neuronal ceroid lipofuscinosis

http://www.wikidata.org/entity/Q34247306

about

Lithium and autophagy Autophagy in the physiology and pathology of the central nervous system The endolysosomal system in cell death and survival A murine model of variant late infantile ceroid lipofuscinosis recapitulates behavioral and pathological phenotypes of human disease A cell-penetrating ester of the neural metabolite lanthionine ketimine stimulates autophagy through the mTORC1 pathway: Evidence for a mechanism of action with pharmacological implications for neurodegenerative pathologies Lysosomal dysfunction and impaired autophagy in a novel mouse model deficient for the lysosomal membrane protein Cln7 Deregulation of subcellular biometal homeostasis through loss of the metal transporter, Zip7, in a childhood neurodegenerative disorder Retinal Degeneration In A Mouse Model Of CLN5 Disease Is Associated With Compromised Autophagy.X-ray fluorescence imaging reveals subcellular biometal disturbances in a childhood neurodegenerative disorder.Induction of lysosomal biogenesis in atherosclerotic macrophages can rescue lipid-induced lysosomal dysfunction and downstream sequelae Strain-dependent brain defects in mouse models of primary ciliary dyskinesia with mutations in Pcdp1 and Spef2 Progressive retinal degeneration and glial activation in the CLN6 (nclf) mouse model of neuronal ceroid lipofuscinosis: a beneficial effect of DHA and curcumin supplementation Altered biometal homeostasis is associated with CLN6 mRNA loss in mouse neuronal ceroid lipofuscinosis Lysosomal membrane permeability stimulates protein aggregate formation in neurons of a lysosomal disease Autophagy in neuronal cells: general principles and physiological and pathological functions.HRES-1/Rab4 promotes the formation of LC3(+) autophagosomes and the accumulation of mitochondria during autophagy.A novel interaction between aging and ER overload in a protein conformational dementia.Dysregulation of autophagy as a common mechanism in lysosomal storage diseases.Murine knockin model for progranulin-deficient frontotemporal dementia with nonsense-mediated mRNA decay.Loss of CLN7 results in depletion of soluble lysosomal proteins and impaired mTOR reactivation.

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P2860

Disruption of the autophagy-lysosome pathway is involved in neuropathology of the nclf mouse model of neuronal ceroid lipofuscinosis

http://www.wikidata.org/entity/Q34247306

description

2012 nî lūn-bûn

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2012 թուականի Ապրիլին հրատարակուած գիտական յօդուած

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2012 թվականի ապրիլին հրատարակված գիտական հոդված

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2012年の論文

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2012年論文

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2012年論文

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2012年論文

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2012年論文

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2012年論文

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2012年论文

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name

Disruption of the autophagy-ly ...... neuronal ceroid lipofuscinosis

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Disruption of the autophagy-ly ...... neuronal ceroid lipofuscinosis

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Disruption of the autophagy-ly ...... neuronal ceroid lipofuscinosis

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Disruption of the autophagy-ly ...... neuronal ceroid lipofuscinosis

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Disruption of the autophagy-ly ...... neuronal ceroid lipofuscinosis

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Disruption of the autophagy-ly ...... neuronal ceroid lipofuscinosis

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Disruption of the autophagy-ly ...... neuronal ceroid lipofuscinosis

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Disruption of the autophagy-ly ...... neuronal ceroid lipofuscinosis

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Disruption of the autophagy-ly ...... neuronal ceroid lipofuscinosis

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Disruption of the autophagy-ly ...... neuronal ceroid lipofuscinosis

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Andrew M S Wong

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Christian Hagel

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Giovanna Galliciotti

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Markus Damme

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Michaela Schweizer

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Thomas Braulke

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P2860

The gene mutated in variant late-infantile neuronal ceroid lipofuscinosis (CLN6) and in nclf mutant mice encodes a novel predicted transmembrane protein

CLN6, which is associated with a lysosomal storage disease, is an endoplasmic reticulum protein

p62/SQSTM1 binds directly to Atg8/LC3 to facilitate degradation of ubiquitinated protein aggregates by autophagy

Defective endoplasmic reticulum-resident membrane protein CLN6 affects lysosomal degradation of endocytosed arylsulfatase A

Mutations in DNAJC5, encoding cysteine-string protein alpha, cause autosomal-dominant adult-onset neuronal ceroid lipofuscinosis

LC3, a mammalian homologue of yeast Apg8p, is localized in autophagosome membranes after processing

Kufs disease, the major adult form of neuronal ceroid lipofuscinosis, caused by mutations in CLN6

Mutations in a novel CLN6-encoded transmembrane protein cause variant neuronal ceroid lipofuscinosis in man and mouse

Autophagy and the ubiquitin-proteasome system: collaborators in neuroprotection

Autophagy in the Pathogenesis of Disease

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e35493

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10.1371/JOURNAL.PONE.0035493

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