Polyglutamine aggregation in Huntington's disease and spinocerebellar ataxia type 3: similar mechanisms in aggregate formation (Q45288723)

11 December 2017

Polyglutamine aggregation in Huntington's disease and spinocerebellar ataxia type 3: similar mechanisms in aggregate formation (English)

1 reference

11 December 2017

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spinocerebellar ataxia

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7

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13 June 2020

Horst Korf

8

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13 June 2020

author name string

K Seidel

1

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13 June 2020

S Siswanto

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13 June 2020

M Fredrich

3

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13 June 2020

M Bouzrou

4

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13 June 2020

E R Brunt

5

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F W van Leeuwen

6

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U Rüb

9

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W F A den Dunnen

10

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publication date

11 June 2015

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Neuropathology and Applied Neurobiology

11 December 2017

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13 June 2020

volume

42

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13 June 2020

issue

2

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13 June 2020

page(s)

153-166

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Protein fate in neurodegenerative proteinopathies: polyglutamine diseases join the (mis)fold

13 June 2020

cites work

1 reference

Polyglutamine neurodegeneration: protein misfolding revisited.

21 January 2018

1 reference

Trinucleotide Repeat Disorders

21 January 2018

1 reference

Analysis of the huntingtin gene reveals a trinucleotide-length polymorphism in the region of the gene that contains two CCG-rich stretches and a correlation between decreased age of onset of Huntington's disease and CAG repeat number

21 January 2018

1 reference

Huntington's disease: from pathology and genetics to potential therapies

21 January 2018

1 reference

Huntington's disease genetics

21 January 2018

1 reference

Degeneration of the cerebellum in Huntington's disease (HD): possible relevance for the clinical picture and potential gateway to pathological mechanisms of the disease process.

21 January 2018

1 reference

The human premotor oculomotor brainstem system - can it help to understand oculomotor symptoms in Huntington's disease?

21 January 2018

1 reference

Huntington's disease (HD): degeneration of select nuclei, widespread occurrence of neuronal nuclear and axonal inclusions in the brainstem

21 January 2018

1 reference

CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1

21 January 2018

1 reference

The deubiquitinating enzyme ataxin-3, a polyglutamine disease protein, edits Lys63 linkages in mixed linkage ubiquitin chains.

21 January 2018

1 reference

The Machado-Joseph disease-associated mutant form of ataxin-3 regulates parkin ubiquitination and stability

21 January 2018

1 reference

Autosomal dominant cerebellar ataxias: clinical features, genetics, and pathogenesis

21 January 2018

1 reference

Brain pathology of spinocerebellar ataxias

21 January 2018

1 reference

Clinical features, neurogenetics and neuropathology of the polyglutamine spinocerebellar ataxias type 1, 2, 3, 6 and 7.

21 January 2018

1 reference

Degeneration of ingestion-related brainstem nuclei in spinocerebellar ataxia type 2, 3, 6 and 7.

21 January 2018

1 reference

Pathoanatomy of cerebellar degeneration in spinocerebellar ataxia type 2 (SCA2) and type 3 (SCA3).

21 January 2018

1 reference

Levels of DNAJB family members (HSP40) correlate with disease onset in patients with spinocerebellar ataxia type 3.

21 January 2018

1 reference

Polyglutamine diseases and transport problems: deadly traffic jams on neuronal highways.

21 January 2018

1 reference

Axonal inclusions in spinocerebellar ataxia type 3.

21 January 2018

1 reference

Protein surveillance machinery in brains with spinocerebellar ataxia type 3: redistribution and differential recruitment of 26S proteasome subunits and chaperones to neuronal intranuclear inclusions

21 January 2018

1 reference

CREB-binding protein sequestration by expanded polyglutamine

21 January 2018

1 reference

Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3.

21 January 2018

1 reference

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

21 January 2018

1 reference

Protein aggregates in Huntington's disease.

21 January 2018

1 reference

Cellular protein quality control and the evolution of aggregates in spinocerebellar ataxia type 3 (SCA3).

21 January 2018

1 reference

Immunohistochemical study of neuronal intranuclear and cytoplasmic inclusions in Machado-Joseph disease.

21 January 2018

1 reference

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

21 January 2018

1 reference

The HSP70 chaperone machinery: J proteins as drivers of functional specificity

21 January 2018

1 reference

The ubiquitin-proteasome pathway: on protein death and cell life

21 January 2018

1 reference

Development by self-digestion: molecular mechanisms and biological functions of autophagy

21 January 2018

1 reference

Protein quality control during aging involves recruitment of the macroautophagy pathway by BAG3

21 January 2018

1 reference

Impairment of proteasome structure and function in aging.

21 January 2018

1 reference

p62/SQSTM1 forms protein aggregates degraded by autophagy and has a protective effect on huntingtin-induced cell death

21 January 2018

1 reference

A DNAJB chaperone subfamily with HDAC-dependent activities suppresses toxic protein aggregation

21 January 2018

1 reference

HspB8, a small heat shock protein mutated in human neuromuscular disorders, has in vivo chaperone activity in cultured cells.

21 January 2018

1 reference

Barcoding heat shock proteins to human diseases: looking beyond the heat shock response

21 January 2018

1 reference

Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias

21 January 2018

1 reference

Frameshift mutants of beta amyloid precursor protein and ubiquitin-B in Alzheimer's and Down patients

21 January 2018

1 reference

Accumulation of aberrant ubiquitin induces aggregate formation and cell death in polyglutamine diseases

21 January 2018

1 reference

Allocortical involvement in Huntington's disease

21 January 2018

1 reference

Dynamic recruitment of active proteasomes into polyglutamine initiated inclusion bodies

21 January 2018

1 reference

PolyQ proteins interfere with nuclear degradation of cytosolic proteins by sequestering the Sis1p chaperone

21 January 2018

1 reference

Oxidative stress and mitochondrial dysfunction in neurodegenerative diseases

21 January 2018

1 reference

21 January 2018

Identifiers

DOI

10.1111/NAN.12253

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26095752%20AND%20SRC:MED&resulttype=core&format=json

13 June 2020

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