Disruption of the Autophagy-Lysosome Pathway Is Involved in Neuropathology of the nclf Mouse Model of Neuronal Ceroid Lipofuscinosis
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Title
Disruption of the Autophagy-Lysosome Pathway Is Involved in Neuropathology of the nclf Mouse Model of Neuronal Ceroid Lipofuscinosis
Authors
Keywords
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Journal
PLoS One
Volume 7, Issue 4, Pages e35493
Publisher
Public Library of Science (PLoS)
Online
2012-04-21
DOI
10.1371/journal.pone.0035493
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Note: Only part of the references are listed.- Mutations in DNAJC5, Encoding Cysteine-String Protein Alpha, Cause Autosomal-Dominant Adult-Onset Neuronal Ceroid Lipofuscinosis
- (2011) Lenka Nosková et al. AMERICAN JOURNAL OF HUMAN GENETICS
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- Pathogenic mutations cause rapid degradation of lysosomal storage disease-related membrane protein CLN6
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- Autophagy and the ubiquitin-proteasome system: Collaborators in neuroprotection
- (2008) Natalia B. Nedelsky et al. BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
- Neuronal ceroid lipofuscinoses
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- Dissecting the ER-Associated Degradation of a Misfolded Polytopic Membrane Protein
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- Autophagy in the Pathogenesis of Disease
- (2008) Beth Levine et al. CELL
- Accumulation of bis(monoacylglycero)phosphate and gangliosides in mouse models of neuronal ceroid lipofuscinosis
- (2008) Sabrina Jabs et al. JOURNAL OF NEUROCHEMISTRY
- Autophagy fights disease through cellular self-digestion
- (2008) Noboru Mizushima et al. NATURE
- Location and connectivity determine GABAergic interneuron survival in the brains of South Hampshire sheep with CLN6 neuronal ceroid lipofuscinosis
- (2008) Manfred J. Oswald et al. NEUROBIOLOGY OF DISEASE
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