(Q36565602)

15 August 2017

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title

The role of ubiquitin-protein ligases in neurodegenerative disease (English)

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15 August 2017

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Helen C Ardley

1

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15 August 2017

Philip A Robinson

series ordinal

2

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15 August 2017

publication date

1 January 2004

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Neurodegenerative Diseases

15 August 2017

published in

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15 August 2017

volume

1

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15 August 2017

issue

2-3

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15 August 2017

page(s)

71-87

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Aberrant protein deposition and neurological disease

15 August 2017

cites work

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Cellular defenses against unfolded proteins: a cell biologist thinks about neurodegenerative diseases

21 January 2018

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Aggresomes: a cellular response to misfolded proteins

21 January 2018

1 reference

Parkin ubiquitinates the alpha-synuclein-interacting protein, synphilin-1: implications for Lewy-body formation in Parkinson disease

21 January 2018

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Transfected synphilin-1 forms cytoplasmic inclusions in HEK293 cells

21 January 2018

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Inducible expression of mutant alpha-synuclein decreases proteasome activity and increases sensitivity to mitochondria-dependent apoptosis

21 January 2018

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Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation

21 January 2018

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Formation and removal of alpha-synuclein aggregates in cells exposed to mitochondrial inhibitors

21 January 2018

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Parkin accumulation in aggresomes due to proteasome impairment

21 January 2018

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Dorfin ubiquitylates mutant SOD1 and prevents mutant SOD1-mediated neurotoxicity

21 January 2018

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Naturally secreted oligomers of amyloid beta protein potently inhibit hippocampal long-term potentiation in vivo

21 January 2018

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Inhibition of proteasomal activity causes inclusion formation in neuronal and non-neuronal cells overexpressing Parkin

21 January 2018

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From neurodegeneration to neurohomeostasis: the role of ubiquitin

21 January 2018

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Protein aggregation and the ubiquitin proteasome pathway: gaining the UPPer hand on neurodegeneration

21 January 2018

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Role of ubiquitin-mediated proteolysis in the pathogenesis of neurodegenerative disorders.

21 January 2018

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Protein folding in vivo: unraveling complex pathways

21 January 2018

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Heat-shock proteins, molecular chaperones, and the stress response: evolutionary and ecological physiology

21 January 2018

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Mechanisms underlying ubiquitination

21 January 2018

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Themes and variations on ubiquitylation

21 January 2018

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The ubiquitin-proteasome pathway of intracellular proteolysis

21 January 2018

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How the ubiquitin-proteasome system controls transcription

21 January 2018

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The N-end rule and regulation of apoptosis

21 January 2018

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Proteolysis, proteasomes and antigen presentation

21 January 2018

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Antigen processing by the proteasome

21 January 2018

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Deubiquitinating enzymes: their diversity and emerging roles

21 January 2018

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Conjugation and deconjugation of ubiquitin regulating the destiny of proteins

21 January 2018

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Protein regulation by monoubiquitin

21 January 2018

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How NF-kappaB is activated: the role of the IkappaB kinase (IKK) complex

21 January 2018

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Hakai, a c-Cbl-like protein, ubiquitinates and induces endocytosis of the E-cadherin complex

21 January 2018

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RING domains: master builders of molecular scaffolds?

21 January 2018

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21 January 2018

RING for destruction?

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The lore of the RINGs: substrate recognition and catalysis by ubiquitin ligases

21 January 2018

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The HPV-16 E6 and E6-AP complex functions as a ubiquitin-protein ligase in the ubiquitination of p53

21 January 2018

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Physical interaction between specific E2 and Hect E3 enzymes determines functional cooperativity

21 January 2018

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Identification of determinants in E2 ubiquitin-conjugating enzymes required for hect E3 ubiquitin-protein ligase interaction

21 January 2018

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Ubiquitination and endocytosis of plasma membrane proteins: role of Nedd4/Rsp5p family of ubiquitin-protein ligases

21 January 2018

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Smad7 binds to Smurf2 to form an E3 ubiquitin ligase that targets the TGF beta receptor for degradation

21 January 2018

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Initial sequencing and analysis of the human genome

21 January 2018

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Proteolysis and the cell cycle: with this RING I do thee destroy

21 January 2018

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RING finger proteins: mediators of ubiquitin ligase activity

21 January 2018

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RING fingers mediate ubiquitin-conjugating enzyme (E2)-dependent ubiquitination

21 January 2018

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The tyrosine kinase negative regulator c-Cbl as a RING-type, E2-dependent ubiquitin-protein ligase

21 January 2018

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Src-catalyzed phosphorylation of c-Cbl leads to the interdependent ubiquitination of both proteins

21 January 2018

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

21 January 2018

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ROC1, a homolog of APC11, represents a family of cullin partners with an associated ubiquitin ligase activity

21 January 2018

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The APC11 RING-H2 finger mediates E2-dependent ubiquitination

21 January 2018

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The RING-H2 finger protein APC11 and the E2 enzyme UBC4 are sufficient to ubiquitinate substrates of the anaphase-promoting complex

21 January 2018

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Identification of the von Hippel-lindau tumor-suppressor protein as part of an active E3 ubiquitin ligase complex

21 January 2018

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Does this have a familiar RING?

21 January 2018

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The RING domains of the promyelocytic leukemia protein PML and the arenaviral protein Z repress translation by directly inhibiting translation initiation factor eIF4E.

21 January 2018

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Interaction of the ring finger-related U-box motif of a nuclear dot protein with ubiquitin-conjugating enzymes

21 January 2018

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U box proteins as a new family of ubiquitin-protein ligases

21 January 2018

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CHIP is a chaperone-dependent E3 ligase that ubiquitylates unfolded protein

21 January 2018

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Structural insights into the U-box, a domain associated with multi-ubiquitination

21 January 2018

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The ubiquitin-protein ligase E6-associated protein (E6-AP) serves as its own substrate

21 January 2018

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Mdm2 is a RING finger-dependent ubiquitin protein ligase for itself and p53

21 January 2018

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A novel centrosomal ring-finger protein, dorfin, mediates ubiquitin ligase activity

21 January 2018

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Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1.

21 January 2018

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CHIP is associated with Parkin, a gene responsible for familial Parkinson's disease, and enhances its ubiquitin ligase activity

21 January 2018

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TorsinA and heat shock proteins act as molecular chaperones: suppression of alpha-synuclein aggregation

21 January 2018

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alpha B crystallin expression in non-lenticular tissues and selective presence in ubiquitinated inclusion bodies in human disease

21 January 2018

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21 January 2018

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Immunohistochemical and subcellular localization of Parkin protein: absence of protein in autosomal recessive juvenile parkinsonism patients

21 January 2018

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Pathological lesions of Alzheimer's disease and dementia with Lewy bodies brains exhibit immunoreactivity to an ATPase that is a regulatory subunit of the 26S proteasome.

21 January 2018

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NACP/alpha-synuclein and tau constitute two distinctive subsets of filaments in the same neuronal inclusions in brains from a family of parkinsonism and dementia with Lewy bodies: double-immunolabeling fluorescence and electron microscopic studies

21 January 2018

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Microtubule-associated protein 1B is a component of cortical Lewy bodies and binds alpha-synuclein filaments

21 January 2018

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Immunohistochemical localization of neurofilament protein in neuronal degenerations.

21 January 2018

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Aggresomes protect cells by enhancing the degradation of toxic polyglutamine-containing protein

21 January 2018

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RING finger 1 mutations in Parkin produce altered localization of the protein

21 January 2018

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Characterization and dynamics of aggresome formation by a cytosolic GFP-chimera

21 January 2018

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Aggresomes, inclusion bodies and protein aggregation

21 January 2018

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Hassles with taking out the garbage: aggravating aggresomes.

21 January 2018

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The Drosophila bendless gene encodes a neural protein related to ubiquitin-conjugating enzymes

21 January 2018

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UBE3A/E6-AP mutations cause Angelman syndrome

21 January 2018

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De novo truncating mutations in E6-AP ubiquitin-protein ligase gene (UBE3A) in Angelman syndrome

21 January 2018

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Alzheimer's disease: Initial report of the purification and characterization of a novel cerebrovascular amyloid protein

21 January 2018

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Cloning of a gene bearing missense mutations in early-onset familial Alzheimer's disease

21 January 2018

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A portrait of Alzheimer secretases--new features and familiar faces

21 January 2018

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Presenilin, Notch, and the genesis and treatment of Alzheimer's disease

21 January 2018

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Expression of Alzheimer's disease-associated presenilin-1 is controlled by proteolytic degradation and complex formation

21 January 2018

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SEL-10 interacts with presenilin 1, facilitates its ubiquitination, and alters A-beta peptide production

21 January 2018

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A rescue factor abolishing neuronal cell death by a wide spectrum of familial Alzheimer's disease genes and Aβ

21 January 2018

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Evidence for in vivo production of Humanin peptide, a neuroprotective factor against Alzheimer's disease-related insults

21 January 2018

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Humanin peptide suppresses apoptosis by interfering with Bax activation

21 January 2018

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A tripartite motif protein TRIM11 binds and destabilizes Humanin, a neuroprotective peptide against Alzheimer's disease-relevant insults

21 January 2018

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Parkinson's disease. First of two parts

21 January 2018

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Etiology and pathogenesis of Parkinson's disease

21 January 2018

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Mutation in the alpha-synuclein gene identified in families with Parkinson's disease

21 January 2018

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Mutations in the parkin gene cause autosomal recessive juvenile parkinsonism

21 January 2018

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The ubiquitin pathway in Parkinson's disease

21 January 2018

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Mutations in the DJ-1 gene associated with autosomal recessive early-onset parkinsonism

21 January 2018

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Mutations in NR4A2 associated with familial Parkinson disease

21 January 2018

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Complete genomic screen in Parkinson disease: evidence for multiple genes.

21 January 2018

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Localization of a novel locus for autosomal recessive early-onset parkinsonism, PARK6, on human chromosome 1p35-p36

21 January 2018

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Ubiquitination of a new form of alpha-synuclein by parkin from human brain: implications for Parkinson's disease

21 January 2018

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Co-association of parkin and alpha-synuclein

21 January 2018

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L166P mutant DJ-1, causative for recessive Parkinson's disease, is degraded through the ubiquitin-proteasome system

21 January 2018

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Association between early-onset Parkinson's disease and mutations in the parkin gene

21 January 2018

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An apparently sporadic case with parkin gene mutation in a Korean woman

21 January 2018

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Mutation screening and association analysis of the parkin gene in Parkinson's disease patients from South-West China.

21 January 2018

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Parkin mutations and susceptibility alleles in late-onset Parkinson's disease

21 January 2018

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Pathologic and biochemical studies of juvenile parkinsonism linked to chromosome 6q.

21 January 2018

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A novel transactivation domain in parkin

21 January 2018

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The ubiquitin-conjugating enzymes UbcH7 and UbcH8 interact with RING finger/IBR motif-containing domains of HHARI and H7-AP1

21 January 2018

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

21 January 2018

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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

21 January 2018

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An unfolded putative transmembrane polypeptide, which can lead to endoplasmic reticulum stress, is a substrate of Parkin

21 January 2018

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Parkin is a component of an SCF-like ubiquitin ligase complex and protects postmitotic neurons from kainate excitotoxicity

21 January 2018

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The p38 subunit of the aminoacyl-tRNA synthetase complex is a Parkin substrate: linking protein biosynthesis and neurodegeneration.

21 January 2018

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SEPT5_v2 is a parkin-binding protein

21 January 2018

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The autosomal recessive juvenile Parkinson disease gene product, parkin, interacts with and ubiquitinates synaptotagmin XI

21 January 2018

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Alpha-synuclein in Lewy bodies

21 January 2018

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Characterization of cytoplasmic alpha-synuclein aggregates. Fibril formation is tightly linked to the inclusion-forming process in cells

21 January 2018

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Parkin protects against the toxicity associated with mutant alpha-synuclein: proteasome dysfunction selectively affects catecholaminergic neurons

21 January 2018

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Parkin suppresses dopaminergic neuron-selective neurotoxicity induced by Pael-R in Drosophila

21 January 2018

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Ubiquitination of alpha-synuclein in Lewy bodies is a pathological event not associated with impairment of proteasome function

21 January 2018

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Dorfin Localizes to Lewy Bodies and Ubiquitylates Synphilin-1

21 January 2018

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Siah-1 facilitates ubiquitination and degradation of synphilin-1

21 January 2018

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

21 January 2018

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Immunocytochemical and ultrastructural studies of lower motor neurons in amyotrophic lateral sclerosis

21 January 2018

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Dorfin localizes to the ubiquitylated inclusions in Parkinson's disease, dementia with Lewy bodies, multiple system atrophy, and amyotrophic lateral sclerosis

21 January 2018

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Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis

21 January 2018

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Misfolded CuZnSOD and amyotrophic lateral sclerosis

21 January 2018

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Mitochondrial dysfunction in a cell culture model of familial amyotrophic lateral sclerosis

21 January 2018

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Mitochondrial dysfunction due to mutant copper/zinc superoxide dismutase associated with amyotrophic lateral sclerosis is reversed by N-acetylcysteine.

21 January 2018

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Proteasomal inhibition by misfolded mutant superoxide dismutase 1 induces selective motor neuron death in familial amyotrophic lateral sclerosis

21 January 2018

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Polyglutamines placed into context

21 January 2018

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Pathogenesis of polyglutamine disorders: aggregation revisited

21 January 2018

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Parkin facilitates the elimination of expanded polyglutamine proteins and leads to preservation of proteasome function.

21 January 2018

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A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes.

21 January 2018

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Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation

21 January 2018

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

21 January 2018

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Huntingtin is ubiquitinated and interacts with a specific ubiquitin-conjugating enzyme

21 January 2018

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The hunt for huntingtin function: interaction partners tell many different stories

21 January 2018

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E3 ligase activity of RING finger proteins that interact with Hip-2, a human ubiquitin-conjugating enzyme

21 January 2018

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Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1.

21 January 2018

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Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures

21 January 2018

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Androgen receptor defects: historical, clinical, and molecular perspectives

21 January 2018

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Nuclear receptor coregulators: cellular and molecular biology

21 January 2018

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The 26S proteasome is required for estrogen receptor-alpha and coactivator turnover and for efficient estrogen receptor-alpha transactivation

21 January 2018

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Differential Regulation of Proteasome-Dependent Estrogen Receptor α and β Turnover in Cultured Human Uterine Artery Endothelial Cells

21 January 2018

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Nuclear inclusions of the androgen receptor protein in spinal and bulbar muscular atrophy

21 January 2018

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Expression of expanded repeat androgen receptor produces neurologic disease in transgenic mice.

21 January 2018

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Transgenic mouse models of spinal and bulbar muscular atrophy (SBMA).

21 January 2018

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Identification of a novel RING finger protein as a coregulator in steroid receptor-mediated gene transcription

21 January 2018

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Cloning and characterization of an androgen receptor N-terminal-interacting protein with ubiquitin-protein ligase activity.

21 January 2018

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Cloning and characterization of human prostate coactivator ARA54, a novel protein that associates with the androgen receptor

21 January 2018

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The multiple nuclear functions of BRCA1: transcription, ubiquitination and DNA repair

21 January 2018

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The Angelman syndrome-associated protein, E6-AP, is a coactivator for the nuclear hormone receptor superfamily

21 January 2018

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Novel triplet repeat containing genes in human brain: cloning, expression, and length polymorphisms

21 January 2018

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Unstable expansion of CAG repeat in hereditary dentatorubral-pallidoluysian atrophy (DRPLA)

21 January 2018

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Drosophila atrophin homolog functions as a transcriptional corepressor in multiple developmental processes

21 January 2018

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Abnormal dentatorubral-pallidoluysian atrophy (DRPLA) protein complex is pathologically ubiquitinated in DRPLA brains

21 January 2018

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Atrophin-1, the DRPLA gene product, interacts with two families of WW domain-containing proteins

21 January 2018

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Polyglutamine expansion, protein aggregation, proteasome activity, and neural survival.

21 January 2018

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The RING heterodimer BRCA1-BARD1 is a ubiquitin ligase inactivated by a breast cancer-derived mutation

21 January 2018

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MdmX is a RING finger ubiquitin ligase capable of synergistically enhancing Mdm2 ubiquitination

21 January 2018

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Enhancement of BRCA1 E3 ubiquitin ligase activity through direct interaction with the BARD1 protein

21 January 2018

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Interaction between two ubiquitin-protein isopeptide ligases of different classes, CBLC and AIP4/ITCH.

21 January 2018

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Shattuck lecture--neurodegenerative diseases and prions

21 January 2018

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Prion proteins meet protein quality control

21 January 2018

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Wild-type PrP and a mutant associated with prion disease are subject to retrograde transport and proteasome degradation

21 January 2018

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Spongiform degeneration in mahoganoid mutant mice

21 January 2018

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Targeted disruption of the Epm2a gene causes formation of Lafora inclusion bodies, neurodegeneration, ataxia, myoclonus epilepsy and impaired behavioral response in mice

21 January 2018

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Genotype-phenotype correlations for EPM2A mutations in Lafora's progressive myoclonus epilepsy: exon 1 mutations associate with an early-onset cognitive deficit subphenotype.

21 January 2018

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Mutations in NHLRC1 cause progressive myoclonus epilepsy

21 January 2018

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Mutations in a gene encoding a novel protein tyrosine phosphatase cause progressive myoclonus epilepsy

21 January 2018

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Ataxin-3, the MJD1 gene product, interacts with the two human homologs of yeast DNA repair protein RAD23, HHR23A and HHR23B

21 January 2018

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The polyglutamine neurodegenerative protein ataxin-3 binds polyubiquitylated proteins and has ubiquitin protease activity

21 January 2018

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Association of ataxin-7 with the proteasome subunit S4 of the 19S regulatory complex

21 January 2018

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SUMO-1 co-localized with mutant atrophin-1 with expanded polyglutamines accelerates intranuclear aggregation and cell death

21 January 2018

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Ubiquitin-binding protein p62 is present in neuronal and glial inclusions in human tauopathies and synucleinopathies

21 January 2018

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Early accumulation of p62 in neurofibrillary tangles in Alzheimer's disease: possible role in tangle formation.

21 January 2018

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Neuronal and glial inclusions in frontotemporal dementia with or without motor neuron disease are immunopositive for p62

21 January 2018

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Proteasomal degradation of tau protein

21 January 2018

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alpha-synuclein metabolism and aggregation is linked to ubiquitin-independent degradation by the proteasome

21 January 2018

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Inhibition of the ubiquitin-proteasome system in Alzheimer's disease.

21 January 2018

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Does an inhibition of the ubiquitin/26S proteasome pathway of protein degradation underlie the pathogenesis of non-familial Alzheimer's disease?

21 January 2018

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Mutant ubiquitin found in neurodegenerative disorders is a ubiquitin fusion degradation substrate that blocks proteasomal degradation

21 January 2018

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21 January 2018

+1 Proteins and aging

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Alzheimer's associated variant ubiquitin causes inhibition of the 26S proteasome and chaperone expression

21 January 2018

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Prevention of β-Amyloid Neurotoxicity by Blockade of the Ubiquitin-Proteasome Proteolytic Pathway

21 January 2018

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21 January 2018

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21 January 2018

Identifiers

DOI

10.1159/000080048

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