Literature DB >> 16282599

The ubiquitin-proteasome system in Huntington's disease.

A G Valera1, M Díaz-Hernández, F Hernández, Z Ortega, J J Lucas.   

Abstract

The main histopathological feature of Huntington's disease (HD) is the presence of protein aggregates that are gathered into inclusion bodies. So far the mechanisms that lead to inclusion formation as well as their role in the pathogenesis of HD are not totally understood. However, it is well established that inclusion bodies contain components of the ubiquitin-proteasome system. Accordingly, it has been postulated that impairment of this machinery can be one of the causes of this disorder. In this review, the authors summarize the state of current knowledge about this hypothesis.

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Year:  2005        PMID： 16282599     DOI： 10.1177/1073858405280639

Source DB:  PubMed          Journal:  Neuroscientist        ISSN： 1073-8584            Impact factor:   7.519


  16 in total

1.  Altered histone monoubiquitylation mediated by mutant huntingtin induces transcriptional dysregulation.

Authors:  Mee-Ohk Kim; Prianka Chawla; Ryan P Overland; Eva Xia; Ghazaleh Sadri-Vakili; Jang-Ho J Cha
Journal:  J Neurosci       Date:  2008-04-09       Impact factor: 6.167

2.  Azadiradione Restores Protein Quality Control and Ameliorates the Disease Pathogenesis in a Mouse Model of Huntington's Disease.

Authors:  Brijesh Kumar Singh; Naman Vatsa; Vinod K Nelson; Vipendra Kumar; Shashi Shekhar Kumar; Subhash C Mandal; Mahadeb Pal; Nihar Ranjan Jana
Journal:  Mol Neurobiol       Date:  2018-01-02       Impact factor: 5.590

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

Authors:  Udo Rüb; Matthias Hentschel; Katharina Stratmann; Ewout Brunt; Helmut Heinsen; Kay Seidel; Mohamed Bouzrou; Georg Auburger; Henry Paulson; Jean-Paul Vonsattel; Herwig Lange; Horst-Werner Korf; Wilfred den Dunnen
Journal:  Brain Pathol       Date:  2014-03-03       Impact factor: 6.508

4.  Shotgun proteomics in neuroscience.

Authors:  Lujian Liao; Daniel B McClatchy; John R Yates
Journal:  Neuron       Date:  2009-07-16       Impact factor: 17.173

Review 5.  Neuroprotection for Huntington's disease: ready, set, slow.

Authors:  Steven M Hersch; H Diana Rosas
Journal:  Neurotherapeutics       Date:  2008-04       Impact factor: 7.620

6.  Indirect inhibition of 26S proteasome activity in a cellular model of Huntington's disease.

Authors:  Mark S Hipp; Chetan N Patel; Kirill Bersuker; Brigit E Riley; Stephen E Kaiser; Thomas A Shaler; Michael Brandeis; Ron R Kopito
Journal:  J Cell Biol       Date:  2012-02-27       Impact factor: 10.539

7.  Mitochondrial permeability transition pore induces mitochondria injury in Huntington disease.

Authors:  Rodrigo A Quintanilla; Youngnam N Jin; Rommy von Bernhardi; Gail V W Johnson
Journal:  Mol Neurodegener       Date:  2013-12-11       Impact factor: 14.195

8.  Adaptive changes in the neuronal proteome: mitochondrial energy production, endoplasmic reticulum stress, and ribosomal dysfunction in the cellular response to metabolic stress.

Authors:  Abigail G Herrmann; Ruth F Deighton; Thierry Le Bihan; Mailis C McCulloch; James L Searcy; Lorraine E Kerr; James McCulloch
Journal:  J Cereb Blood Flow Metab       Date:  2013-01-16       Impact factor: 6.200

Review 9.  The ubiquitin proteasome system in Huntington's disease and the spinocerebellar ataxias.

Authors:  Janet E Davies; Sovan Sarkar; David C Rubinsztein
Journal:  BMC Biochem       Date:  2007-11-22       Impact factor: 4.059

10.  PiZ mouse liver accumulates polyubiquitin conjugates that associate with catalytically active 26S proteasomes.

Authors:  Christopher J Haddock; Keith Blomenkamp; Madhav Gautam; Jared James; Joanna Mielcarska; Edward Gogol; Jeffrey Teckman; Dorota Skowyra
Journal:  PLoS One       Date:  2014-09-11       Impact factor: 3.240
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