(Q28269322)

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The roles of intracellular protein-degradation pathways in neurodegeneration

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Europe PubMed Central

The roles of intracellular protein-degradation pathways in neurodegeneration (English)

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neurodegeneration

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David C Rubinsztein

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19 October 2006

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780-6

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Toxic proteins in neurodegenerative disease

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Opinion: What is the role of protein aggregation in neurodegeneration?

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Cause of neural death in neurodegenerative diseases attributable to expansion of glutamine repeats

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Disease modifying strategies for the treatment of Alzheimer's disease targeted at modulating levels of the beta-amyloid peptide

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Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease.

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Cdk5 phosphorylation of huntingtin reduces its cleavage by caspases: implications for mutant huntingtin toxicity

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Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract

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The ubiquitin proteolytic system: from a vague idea, through basic mechanisms, and onto human diseases and drug targeting

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A series of ubiquitin binding factors connects CDC48/p97 to substrate multiubiquitylation and proteasomal targeting

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Inclusion body myopathy-associated mutations in p97/VCP impair endoplasmic reticulum-associated degradation

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7 January 2021

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Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy

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α-Synuclein Is Degraded by Both Autophagy and the Proteasome

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Aggregate formation inhibits proteasomal degradation of polyglutamine proteins.

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Eukaryotic proteasomes cannot digest polyglutamine sequences and release them during degradation of polyglutamine-containing proteins

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Inefficient degradation of truncated polyglutamine proteins by the proteasome

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Autophagy: molecular machinery for self-eating

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Regulation of intracellular accumulation of mutant Huntingtin by Beclin 1

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Increased susceptibility of cytoplasmic over nuclear polyglutamine aggregates to autophagic degradation

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Autophagy regulates the processing of amino terminal huntingtin fragments

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Rapamycin alleviates toxicity of different aggregate-prone proteins

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Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease

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Impairment of starvation-induced and constitutive autophagy in Atg7-deficient mice

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Raised intracellular glucose concentrations reduce aggregation and cell death caused by mutant huntingtin exon 1 by decreasing mTOR phosphorylation and inducing autophagy

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7 January 2021

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Involvement of macroautophagy in the dissolution of neuronal inclusions.

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Pathophysiology of chaperone-mediated autophagy

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Altered proteasomal function in sporadic Parkinson's disease

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Generalized brain and skin proteasome inhibition in Huntington's disease

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Impairment of the ubiquitin-proteasome system by protein aggregation

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Neuronal dysfunction in a polyglutamine disease model occurs in the absence of ubiquitin-proteasome system impairment and inversely correlates with the degree of nuclear inclusion formation

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7 January 2021

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Neuronal induction of the immunoproteasome in Huntington's disease.

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Caspase activation inhibits proteasome function during apoptosis

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7 January 2021

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Parkin functions as an E2-dependent ubiquitin- protein ligase and promotes the degradation of the synaptic vesicle-associated protein, CDCrel-1

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7 January 2021

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Familial Parkinson disease gene product, parkin, is a ubiquitin-protein ligase

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Parkin suppresses unfolded protein stress-induced cell death through its E3 ubiquitin-protein ligase activity

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The ubiquitin pathway in Parkinson's disease

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Molecular pathophysiology of Parkinson's disease

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UCHL-1 is not a Parkinson's disease susceptibility gene

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7 January 2021

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The UCH-L1 gene encodes two opposing enzymatic activities that affect alpha-synuclein degradation and Parkinson's disease susceptibility

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7 January 2021

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Intragenic deletion in the gene encoding ubiquitin carboxy-terminal hydrolase in gad mice

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7 January 2021

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Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein

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7 January 2021

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Systemic exposure to proteasome inhibitors causes a progressive model of Parkinson's disease

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The role of autophagy during the early neonatal starvation period

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Inhibition of macroautophagy triggers apoptosis

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Rapamycin pre-treatment protects against apoptosis

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Loss of autophagy in the central nervous system causes neurodegeneration in mice

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Suppression of basal autophagy in neural cells causes neurodegenerative disease in mice

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Dynein mutations impair autophagic clearance of aggregate-prone proteins

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A motor neuron disease-associated mutation in p150Glued perturbs dynactin function and induces protein aggregation

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Mutant dynactin in motor neuron disease.

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Mutations in dynein link motor neuron degeneration to defects in retrograde transport

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A mutation in dynein rescues axonal transport defects and extends the life span of ALS mice

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Mutant superoxide dismutase disrupts cytoplasmic dynein in motor neurons

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Impaired degradation of mutant alpha-synuclein by chaperone-mediated autophagy

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https://api.crossref.org/works/10.1038%2FNATURE05291

7 January 2021

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RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model

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Allele-specific RNA interference for neurological disease

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Trehalose reduces aggregate formation and delays pathology in a transgenic mouse model of oculopharyngeal muscular dystrophy

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Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders.

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Proteasomal-dependent aggregate reversal and absence of cell death in a conditional mouse model of Huntington's disease.

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Autophagy-mediated clearance of huntingtin aggregates triggered by the insulin-signaling pathway

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7 January 2021

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Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain

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Oxidative stress and autophagy

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Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death

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Sirolimus: its role in nephrology.

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The TOR pathway: a target for cancer therapy

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Phase II trial of temsirolimus (CCI-779) in recurrent glioblastoma multiforme: a North Central Cancer Treatment Group Study

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TOR signaling in growth and metabolism

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Lithium induces autophagy by inhibiting inositol monophosphatase

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How understanding the control of energy metabolism can help investigation of mitochondrial dysfunction, regulation and pharmacology

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Essential roles of Atg5 and FADD in autophagic cell death: dissection of autophagic cell death into vacuole formation and cell death

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7 January 2021

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Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes

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7 January 2021

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Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue

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7 January 2021

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Inactivation of the mouse Huntington's disease gene homolog Hdh

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Lessons from animal models of Huntington's disease

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Ectopically expressed CAG repeats cause intranuclear inclusions and a progressive late onset neurological phenotype in the mouse

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7 January 2021

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Normal huntingtin function: an alternative approach to Huntington's disease

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7 January 2021

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