Huntington's disease: degradation of mutant huntingtin by autophagy.
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Ubiquitin-proteasome system involvement in Huntington's diseaseLaforin, the most common protein mutated in Lafora disease, regulates autophagyIntrabodies as neuroprotective therapeuticsMaintenance of basal levels of autophagy in Huntington's disease mouse models displaying metabolic dysfunctionPotential therapeutic effects of the MTOR inhibitors for preventing ageing and progeria-related disordersTherapeutic Targeting of AutophagyAMPK and mTOR regulate autophagy through direct phosphorylation of Ulk1A high-throughput-compatible assay to measure the degradation of endogenous Huntingtin proteinsDeletion of the huntingtin polyglutamine stretch enhances neuronal autophagy and longevity in mice.Macroautophagy deficiency mediates age-dependent neurodegeneration through a phospho-tau pathway.mTOR regulates tau phosphorylation and degradation: implications for Alzheimer's disease and other tauopathies.Identification of a post-translationally myristoylated autophagy-inducing domain released by caspase cleavage of huntingtin.The regulation of N-terminal Huntingtin (Htt552) accumulation by Beclin1.The vacuole import and degradation pathway utilizes early steps of endocytosis and actin polymerization to deliver cargo proteins to the vacuole for degradationEarly AMD-like defects in the RPE and retinal degeneration in aged mice with RPE-specific deletion of Atg5 or Atg7The PI3K/Akt/mTOR pathway is involved in CVB3-induced autophagy of HeLa cells.Cargo recognition failure is responsible for inefficient autophagy in Huntington's disease.Rapamycin prevents the mutant huntingtin-suppressed GLT-1 expression in cultured astrocytes.Inhibiting the ubiquitin-proteasome system leads to preferential accumulation of toxic N-terminal mutant huntingtin fragmentsThe interplay of turn formation and hydrophobic interactions on the early kinetic events in protein folding.Early autophagic response in a novel knock-in model of Huntington diseaseBifunctional anti-huntingtin proteasome-directed intrabodies mediate efficient degradation of mutant huntingtin exon 1 protein fragments.Mechanical injury suppresses autophagy regulators and pharmacologic activation of autophagy results in chondroprotection.AUTEN-67 (Autophagy Enhancer-67) Hampers the Progression of Neurodegenerative Symptoms in a Drosophila model of Huntington's Disease.Type 2 transglutaminase in Huntington's disease: a double-edged sword with clinical potential.Regulation of autophagy by mTOR-dependent and mTOR-independent pathways: autophagy dysfunction in neurodegenerative diseases and therapeutic application of autophagy enhancers.Development of an ELISA assay for the quantification of soluble huntingtin in human blood cells.Molecular characterization of skeletal muscle atrophy in the R6/2 mouse model of Huntington's diseaseGene therapy in mouse models of huntington disease.Degradation of misfolded proteins in neurodegenerative diseases: therapeutic targets and strategies.Autophagy activation by rapamycin reduces severity of experimental osteoarthritis.Answering the ultimate question "what is the proximal cause of aging?"Rapamycin reduces intrahepatic alpha-1-antitrypsin mutant Z protein polymers and liver injury in a mouse modelCompartment-Dependent Degradation of Mutant Huntingtin Accounts for Its Preferential Accumulation in Neuronal ProcessesCytoplasmic retention of polyglutamine-expanded androgen receptor ameliorates disease via autophagy in a mouse model of spinal and bulbar muscular atrophy.Tragedy in a heartbeat: malfunctioning desmin causes skeletal and cardiac muscle diseaseA triazole derivative elicits autophagic clearance of polyglutamine aggregation in neuronal cells.The cellular pathways of neuronal autophagy and their implication in neurodegenerative diseases.Regulation of amyloid precursor protein processing by its KFERQ motif.Elevated ATG5 expression in autoimmune demyelination and multiple sclerosis.
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Huntington's disease: degradation of mutant huntingtin by autophagy.
description
article científic
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article scientifique
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articolo scientifico
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artigo científico
@pt
bilimsel makale
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scientific article published on 15 July 2008
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vedecký článok
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vetenskaplig artikel
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videnskabelig artikel
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vědecký článek
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name
Huntington's disease: degradation of mutant huntingtin by autophagy.
@en
Huntington's disease: degradation of mutant huntingtin by autophagy.
@nl
type
label
Huntington's disease: degradation of mutant huntingtin by autophagy.
@en
Huntington's disease: degradation of mutant huntingtin by autophagy.
@nl
prefLabel
Huntington's disease: degradation of mutant huntingtin by autophagy.
@en
Huntington's disease: degradation of mutant huntingtin by autophagy.
@nl
P2860
P1433
P1476
Huntington's disease: degradation of mutant huntingtin by autophagy.
@en
P2093
David C Rubinsztein
Sovan Sarkar
P2860
P304
P356
10.1111/J.1742-4658.2008.06562.X
P407
P577
2008-07-15T00:00:00Z