Stephen W. Davies
University College London
34 Papers
500 Citations
Stephen W. Davies is an academic researcher from University College London. The author has contributed to research in topics: Huntington's disease & Neurodegeneration. The author has an hindex of 26, co-authored 33 publications. Previous affiliations of Stephen W. Davies include Guy's Hospital & University of Southampton.
Chat about Author
Papers
Exon 1 of the HD Gene with an Expanded CAG Repeat Is Sufficient to Cause a Progressive Neurological Phenotype in Transgenic Mice
Laura Mangiarini,Kirupa Sathasivam,Mary J. Seller,Barbara A. Cozens,Alex Harper,Colin Hetherington,Martin Lawton,Yvon Trottier,Hans Lehrach,Stephen W. Davies,Gillian P. Bates +10 more
TL;DR: Mice have been generated that are transgenic for the 5' end of the human HD gene carrying CAG/polyglutamine repeat expansion that exhibits many of the features of HD, including choreiform-like movements, involuntary stereotypic movements, tremor, and epileptic seizures.
3.2K
Aggregation of Huntingtin in Neuronal Intranuclear Inclusions and Dystrophic Neurites in Brain
Marian DiFiglia,Ellen Sapp,Kathryn Chase,Stephen W. Davies,Gillian P. Bates,J. P. Vonsattel,Neil Aronin +6 more
TL;DR: An NH2-terminal fragment of mutant huntingtin was localized to neuronal intranuclear inclusions and dystrophic neurites in the HD cortex and striatum, and polyglutamine length influenced the extent of huntingtin accumulation in these structures.
2.9K
Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the hd mutation
Stephen W. Davies,Mark Turmaine,Barbara A. Cozens,Marian DiFiglia,Alan H. Sharp,Christopher A. Ross,Eberhard Scherzinger,Erich E. Wanker,Laura Mangiarini,Gillian P. Bates +9 more
TL;DR: In this paper, the authors observed that mice transgenic for exon 1 of the human HD gene carrying (CAG)115 to 157 repeat expansions develop pronounced neuronal intranuclear inclusions, containing the proteins huntingtin and ubiquitin, prior to developing a neurological phenotype.
2.3K
Huntingtin-Encoded Polyglutamine Expansions Form Amyloid-like Protein Aggregates In Vitro and In Vivo
Eberhard Scherzinger,Rudi Lurz,Mark Turmaine,Laura Mangiarini,Birgit Hollenbach,Renate Hasenbank,Gillian P. Bates,Stephen W. Davies,Hans Lehrach,Erich E. Wanker +9 more
TL;DR: In this study, it is shown that the proteolytic cleavage of a GST-huntingtin fusion protein leads to the formation of insoluble high molecular weight protein aggregates only when the polyglutamine expansion is in the pathogenic range.
1.3K
Altered neurotransmitter receptor expression in transgenic mouse models of Huntington's disease
Jang-Ho J. Cha,Ariel S. Frey,Stephen A. Alsdorf,J A Kerner,Christoph M. Kosinski,Laura Mangiarini,John B. Penney,Stephen W. Davies,Gillian P. Bates,Anne B. Young +9 more
TL;DR: The results suggest that receptor decreases precede, and therefore might contribute to, the development of clinical symptoms, and altered transcription of specific genes might be a key pathological mechanism in HD.
260