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Ewout Brunt

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Udo Rüb; Kay Seidel; Charlotte von Gall; Jean-Paul Vonsattel; Jürgen Bohl; Ewout R Brunt; Domenico Del Turco; Helmut Heinsen; Franziska Hoche; Horst-Werner Korf; Henry L Paulson; Wilfred F A den Dunnen
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.
Kay Seidel; F W van Leeuwen; J Vinet; M P Zijlstra; G J Dugbartey; H H Kampinga; M Meister; Udo Rüb; Ewout R Brunt; Wilfred F A den Dunnen
Cellular protein quality control and the evolution of aggregates in spinocerebellar ataxia type 3 (SCA3).
W Scherzed; Ludger Schöls; Kay Seidel; Georg Auburger; Ewout R Brunt; Katrin Bürk; Rob A I de Vos; Domenico Del Turco; Thomas Deller; Wilfred F A den Dunnen; Helmut Heinsen; Horst-Werner Korf; Udo Rüb
Pathoanatomy of cerebellar degeneration in spinocerebellar ataxia type 2 (SCA2) and type 3 (SCA3).

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All Publications

2013: Udo Rüb; Kay Seidel; Charlotte von Gall; Jean-Paul Vonsattel; Jürgen Bohl; Ewout R Brunt; Domenico Del Turco; Helmut Heinsen; Franziska Hoche; Horst-Werner Korf; Henry L Paulson; Wilfred F A den Dunnen
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.
Brain pathology (Zurich, Switzerland) 2013;23(2):165-77.
2012: Kay Seidel; F W van Leeuwen; J Vinet; M P Zijlstra; G J Dugbartey; H H Kampinga; M Meister; Udo Rüb; Ewout R Brunt; Wilfred F A den Dunnen
Cellular protein quality control and the evolution of aggregates in spinocerebellar ataxia type 3 (SCA3).
Neuropathology and applied neurobiology 2012;38(6):548-58.
2012: W Scherzed; Ludger Schöls; Kay Seidel; Georg Auburger; Ewout R Brunt; Katrin Bürk; Rob A I de Vos; Domenico Del Turco; Thomas Deller; Wilfred F A den Dunnen; Helmut Heinsen; Horst-Werner Korf; Udo Rüb
Pathoanatomy of cerebellar degeneration in spinocerebellar ataxia type 2 (SCA2) and type 3 (SCA3).
Cerebellum (London, England) 2012;11(3):749-60.
2012: Kay Seidel; Sonny Siswanto; Ewout R Brunt; Wilfred F A den Dunnen; Horst-Werner Korf; Udo Rüb
Brain pathology of spinocerebellar ataxias.
Acta neuropathologica 2012;124(1):1-21.
2011: Deborah A Sival; Maria E Pouwels; Agnes Van Brederode; Natasha Maurits; Corien C Verschuuren-Bemelmans; Ewout R Brunt; Gideon J Du Marchie Sarvaas; Renate J Verbeek; Oebele F Brouwer; Johannes H Van Der Hoeven
In children with Friedreich ataxia, muscle and ataxia parameters are associated.
Developmental medicine and child neurology 2011;53(6):529-34.
2010: Michel J Vos; Marianne P Zijlstra; Bart Kanon; Maria A W H van Waarde-Verhagen; Ewout R Brunt; Hendrika M J Oosterveld-Hut; Serena Carra; Ody C M Sibon; Harm H Kampinga
HSPB7 is the most potent polyQ aggregation suppressor within the HSPB family of molecular chaperones.
Human molecular genetics 2010;19(23):4677-93.
2010: Kay Seidel; Wilfred F A den Dunnen; Christian Schultz; Henry L Paulson; Stefanie Frank; Rob A I de Vos; Ewout R Brunt; Thomas Deller; Harm H Kampinga; Udo Rüb
Axonal inclusions in spinocerebellar ataxia type 3.
Acta neuropathologica 2010;120(4):449-60.
2010: M P Zijlstra; María A Rujano; Maria A W H van Waarde; E Vis; Ewout R Brunt; Harm H Kampinga
Levels of DNAJB family members (HSP40) correlate with disease onset in patients with spinocerebellar ataxia type 3.
The European journal of neuroscience 2010;32(5):760-70.
2009: Kristin Gierga; H J Schelhaas; Ewout R Brunt; Kay Seidel; W Scherzed; R Egensperger; Rob A I de Vos; W den Dunnen; PF Ippel; E Petrasch-Parwez; Thomas Deller; Ludger Schöls; Udo Rüb
Spinocerebellar ataxia type 6 (SCA6): neurodegeneration goes beyond the known brain predilection sites.
Neuropathology and applied neurobiology 2009;35(5):515-27.
2009: M M M Verhagen; W Farid Abdo; Michèl A A P Willemsen; F B L Hogervorst; Dominique Smeets; J A P Hiel; Ewout R Brunt; M A van Rijn; D Majoor Krakauer; R A Oldenburg; Annegien Broeks; J I Last; Laura J van't Veer; M A J Tijssen; A M I Dubois; H P H Kremer; Corry M R Weemaes; A M R Taylor; Marcel van Deuren
Clinical spectrum of ataxia-telangiectasia in adulthood.
Neurology 2009;73(6):430-7.
2009: Deborah A Sival; Ewout R Brunt
The International Cooperative Ataxia Rating Scale shows strong age-dependency in children.
Developmental medicine and child neurology 2009;51(7):571-2.
2009: Udo Rüb; Helmut Heinsen; Ewout R Brunt; B Landwehrmeyer; W F A Den Dunnen; Kristin Gierga; Thomas Deller
The human premotor oculomotor brainstem system - can it help to understand oculomotor symptoms in Huntington's disease?
Neuropathology and applied neurobiology 2009;35(1):4-15.
2008: F Hoche; Kay Seidel; Ewout R Brunt; Georg Auburger; Ludger Schöls; Katrin Bürk; Rob A I de Vos; W den Dunnen; I Bechmann; R Egensperger; Christine Van Broeckhoven; Kristin Gierga; Thomas Deller; Udo Rüb
Involvement of the auditory brainstem system in spinocerebellar ataxia type 2 (SCA2), type 3 (SCA3) and type 7 (SCA7).
Neuropathology and applied neurobiology 2008;34(5):479-91.
2008: Udo Rüb; Ewout R Brunt; Thomas Deller
New insights into the pathoanatomy of spinocerebellar ataxia type 3 (Machado-Joseph disease).
Current opinion in neurology 2008;21(2):111-6.
2008: Udo Rüb; Ewout R Brunt; Kay Seidel; Kristin Gierga; C M Mooy; M Kettner; Christine Van Broeckhoven; I Bechmann; A R La Spada; Ludger Schöls; W den Dunnen; Rob A I de Vos; Thomas Deller
Spinocerebellar ataxia type 7 (SCA7): widespread brain damage in an adult-onset patient with progressive visual impairments in comparison with an adult-onset patient without visual impairments.
Neuropathology and applied neurobiology 2008;34(2):155-68.
2007: Sandra Martins; Francesc Calafell; Claudia Gaspar; Virginia C N Wong; Isabel Silveira; Garth A Nicholson; Ewout R Brunt; Lisbeth Tranebjaerg; Giovanni Stevanin; Mingli Hsieh; bing-wen soong; Leal Loureiro; Alexandra Dürr; Shoji Tsuji; Mitsunori Watanabe; Laura B Jardim; Paola Giunti; Olaf Riess; Laura P W Ranum; Alexis Brice; Guy Rouleau; Paula Coutinho; António Amorim; Jorge Sequeiros
Asian origin for the worldwide-spread mutational event in Machado-Joseph disease.
Archives of neurology 2007;64(10):1502-8.
2007: Udo Rüb; Kay Seidel; Inci Ozerden; Kristin Gierga; Ewout R Brunt; Ludger Schöls; Rob A I de Vos; Wilfred den Dunnen; Christian Schultz; Georg Auburger; Thomas Deller
Consistent affection of the central somatosensory system in spinocerebellar ataxia type 2 and type 3 and its significance for clinical symptoms and rehabilitative therapy.
Brain research reviews 2007;53(2):235-49.
2006: María A Rujano; Floris Bosveld; Florian A Salomons; Freark Dijk; Maria A W H van Waarde; Johannes van der Want; Rob A I de Vos; Ewout R Brunt; Ody C M Sibon; Harm H Kampinga
Polarised asymmetric inheritance of accumulated protein damage in higher eukaryotes.
PLoS biology 2006;4(12):e417.
2006: Udo Rüb; Ewout R Brunt; E Petrasch-Parwez; Ludger Schöls; D Theegarten; Georg Auburger; Kay Seidel; Christian Schultz; Kristin Gierga; Henry L Paulson; Christine Van Broeckhoven; Thomas Deller; Rob A I de Vos
Degeneration of ingestion-related brainstem nuclei in spinocerebellar ataxia type 2, 3, 6 and 7.
Neuropathology and applied neurobiology 2006;32(6):635-49.
2006: Bernd Evert; J Schelhaas; H Fleischer; Rob A I de Vos; Ewout R Brunt; W Stenzel; Thomas Klockgether; Ullrich Wüllner
Neuronal intranuclear inclusions, dysregulation of cytokine expression and cell death in spinocerebellar ataxia type 3.
Clinical neuropathology 2006;25(6):272-81.
2006: Udo Rüb; Rob A I de Vos; Ewout R Brunt; Tamás Sebestény; Ludger Schöls; Georg Auburger; Jürgen R E Bohl; Estifanos Ghebremedhin; Kristin Gierga; Kay Seidel; Wilfred den Dunnen; Helmut Heinsen; Henry L Paulson; Thomas Deller
Spinocerebellar ataxia type 3 (SCA3): thalamic neurodegeneration occurs independently from thalamic ataxin-3 immunopositive neuronal intranuclear inclusions.
Brain pathology (Zurich, Switzerland) 2006;16(3):218-27.
2005: Udo Rüb; Kristin Gierga; Ewout R Brunt; Rob A I de Vos; M Bauer; Ludger Schöls; Katrin Bürk; Georg Auburger; Jürgen R E Bohl; Christian Schultz; Mario Vuksic; Guido J Burbach; Heiko Braak; Thomas Deller
Spinocerebellar ataxias types 2 and 3: degeneration of the pre-cerebellar nuclei isolates the three phylogenetically defined regions of the cerebellum.
Journal of neural transmission (Vienna, Austria : 1996) 2005;112(11):1523-45.
2005: Udo Rüb; Ewout R Brunt; Kristin Gierga; Kay Seidel; Christian Schultz; Ludger Schöls; Georg Auburger; Helmut Heinsen; PF Ippel; W F Glimmerveen; D Wittebol-Post; K Arai; Thomas Deller; Rob A I de Vos
Spinocerebellar ataxia type 7 (SCA7): first report of a systematic neuropathological study of the brain of a patient with a very short expanded CAG-repeat.
Brain pathology (Zurich, Switzerland) 2005;15(4):287-95.
2004: Udo Rüb; Katrin Bürk; Ludger Schöls; Ewout R Brunt; Rob A I de Vos; G Orozco Diaz; Kristin Gierga; Estifanos Ghebremedhin; Christian Schultz; Domenico Del Turco; M Mittelbronn; Georg Auburger; Thomas Deller; Heiko Braak
Damage to the reticulotegmental nucleus of the pons in spinocerebellar ataxia type 1, 2, and 3.
Neurology 2004;63(7):1258-63.
2004: Remko de Pril; David F Fischer; Marion L C Maat-Schieman; Barbara Hobo; Rob A I de Vos; Ewout R Brunt; Elly M Hol; Raymund A C Roos; Fijs W B van Leeuwen
Accumulation of aberrant ubiquitin induces aggregate formation and cell death in polyglutamine diseases.
Human molecular genetics 2004;13(16):1803-13.
2004: Udo Rüb; Ewout R Brunt; Rob A I de Vos; Domenico Del Turco; Kelly Del Tredici; Kristin Gierga; Christian Schultz; Estifanos Ghebremedhin; Katrin Bürk; Georg Auburger; Heiko Braak
Degeneration of the central vestibular system in spinocerebellar ataxia type 3 (SCA3) patients and its possible clinical significance.
Neuropathology and applied neurobiology 2004;30(4):402-14.
2004: Sarah J Shoesmith Berke; Francisca A Flores Schmied; Ewout R Brunt; Lisa M Ellerby; Henry L Paulson
Caspase-mediated proteolysis of the polyglutamine disease protein ataxin-3.
Journal of neurochemistry 2004;89(4):908-18.
2003: Bernd Evert; Ina R Vogt; Ana M Vieira-Saecker; Lucia Ozimek; Rob A I de Vos; Ewout R Brunt; Thomas Klockgether; Ullrich Wüllner
Gene expression profiling in ataxin-3 expressing cell lines reveals distinct effects of normal and mutant ataxin-3.
Journal of neuropathology and experimental neurology 2003;62(10):1006-18.
2003: Udo Rüb; Domenico Del Turco; Kelly Del Tredici; Rob A I de Vos; Ewout R Brunt; G Reifenberger; Carola Seifried; Christian Schultz; Georg Auburger; Heiko Braak
Thalamic involvement in a spinocerebellar ataxia type 2 (SCA2) and a spinocerebellar ataxia type 3 (SCA3) patient, and its clinical relevance.
Brain : a journal of neurology 2003;126(Pt 10):2257-72.
2003: Udo Rüb; Ewout R Brunt; Kristin Gierga; Christian Schultz; Henry L Paulson; Rob A I de Vos; Heiko Braak
The nucleus raphe interpositus in spinocerebellar ataxia type 3 (Machado-Joseph disease).
Journal of chemical neuroanatomy 2003;25(2):115-27.
2003: Udo Rüb; Ewout R Brunt; Domenico Del Turco; Rob A I de Vos; Kristin Gierga; Henry L Paulson; Heiko Braak
Guidelines for the pathoanatomical examination of the lower brain stem in ingestive and swallowing disorders and its application to a dysphagic spinocerebellar ataxia type 3 patient.
Neuropathology and applied neurobiology 2003;29(1):1-13.
2002: Udo Rüb; Rob A I de Vos; Ewout R Brunt; Christian Schultz; Henry L Paulson; Kelly Del Tredici; Heiko Braak
Degeneration of the external cuneate nucleus in spinocerebellar ataxia type 3 (Machado-Joseph disease).
Brain research 2002;953(1-2):126-34.
2002: Udo Rüb; Rob A I de Vos; Christian Schultz; Ewout R Brunt; Henry L Paulson; Heiko Braak
Spinocerebellar ataxia type 3 (Machado-Joseph disease): severe destruction of the lateral reticular nucleus.
Brain : a journal of neurology 2002;125(Pt 9):2115-24.
2002: Bart P C van de Warrenburg; Richard J Sinke; C C Verschuuren-Bemelmans; Hans Scheffer; Ewout R Brunt; PF Ippel; JA Maat-Kievit; Dennis Dooijes; Nicolette C Notermans; Dick Lindhout; Nine V A M Knoers; H P H Kremer
Spinocerebellar ataxias in the Netherlands: prevalence and age at onset variance analysis.
Neurology 2002;58(5):702-8.
2001: Kathryn J Swoboda; bing-wen soong; Catherine E McKenna; Ewout R Brunt; M Litt; J F Bale; T Ashizawa; LB Bennett; Anne M Bowcock; E Steve Roach; D Gerson; T Matsuura; P T Heydemann; M P Nespeca; Joseph Jankovic; M Leppert; LJ Ptácek
Paroxysmal kinesigenic dyskinesia and infantile convulsions. Clinical and linkage studies. 2000.
Neurology 2001;57(11 Suppl 4):S42-8.
2001: Bernd Evert; Ina R Vogt; C Kindermann; L Ozimek; Rob A I de Vos; Ewout R Brunt; Ina Schmitt; Thomas Klockgether; Ullrich Wüllner
Inflammatory genes are upregulated in expanded ataxin-3-expressing cell lines and spinocerebellar ataxia type 3 brains.
The Journal of neuroscience : the official journal of the Society for Neuroscience 2001;21(15):5389-96.
2000: Kathryn J Swoboda; bing-wen soong; Catherine E McKenna; Ewout R Brunt; M Litt; J F Bale; T Ashizawa; LB Bennett; Anne M Bowcock; E Steve Roach; D Gerson; T Matsuura; P T Heydemann; M P Nespeca; Joseph Jankovic; M Leppert; LJ Ptácek
Paroxysmal kinesigenic dyskinesia and infantile convulsions: clinical and linkage studies.
Neurology 2000;55(2):224-30.
1998: Hans Scheffer; Ewout R Brunt; G J Mol; Pieter van der Vlies; Rein P Stulp; Edwin Verlind; G Mantel; Y N Averyanov; Robert M W Hofstra; Charles H C M Buys
Three novel KCNA1 mutations in episodic ataxia type I families.
Human genetics 1998;102(4):464-6.