2 results
11 - Orchestral Works in Performance
-
- By Thierry Fischer, Music Director of the Utah Symphony
- Edited by David Charlton
-
- Book:
- The Music of Simon Holt
- Published by:
- Boydell & Brewer
- Published online:
- 01 September 2018
- Print publication:
- 30 September 2017, pp 232-242
-
- Chapter
- Export citation
-
Summary
EDITOR's INTRODUCTION
THIERRY FISCHER, the Swiss conductor, has to date programmed fourteen pieces by Simon Holt in various concerts over three continents. Of these fourteen, seven have also had their world premiere under his direction. Orchestras involved have been the Nagoya Philharmonic Orchestra (Japan), the Essen Philharmoniker and the Ensemble Modern (Germany), Utah Symphony Orchestra (USA), BBC National Orchestra of Wales, London Sinfonietta and the Royal Academy Symphony Orchestra. Fischer's earliest performances of Holt's orchestral music were in November 1998 when directing 3 for Icarus at the Huddersfield Contemporary Music Festival, when Zoë Martlew played solo cello.
The following texts are the result of email correspondence over twelve months. They have been re-ordered for publication so as to follow the chronological sequence of composition of the music discussed (seven pieces in all). This method of compilation permitted the involvement of others: thus, besides the editor (DC), Edward Venn (EV), Steph Power (SP) and David Beard (DB) offered the questions relative to those pieces about which they were writing in their respective chapters. Thierry Fischer's responses were later revised by him for publication.
QUESTIONS AND RESPONSES
DC: Is it possible to know an orchestra's opinion of the music they play, Simon's in particular?
TF: One particularity of Simon's music is that he writes, at the same time, very challenging and unbelievably simple music. This then immediately opens unexpected doors right from the first play-through. I remember that almost every time I conduct his music, I first perceive incredulity in the eyes of the musicians in front of me. One of the reasons is that the only thing Simon is listening to when he writes music is his deep urge to express beauty and/or pain, without looking at any method, trend, vogue, style or fashion. My experience is that players are often, at first, disturbed by Simon's music because he is bringing them to areas where the notion of beauty and simplicity isn't definable. After a few rehearsals, generally most of the musicians recognise they've gone through an experience they hadn't anticipated. Finally, I believe that the biggest strength of Simon's music is that musicians don't necessarily have an ‘opinion’ about his music. They have much more: a musical experience, creating possibilities in a world of impossibilities.
Cyclin-Dependent Kinase-Like 5 (CDKL5) Mutation Screening in Rett Syndrome and Related Disorders
- Rose White, Gladys Ho, Swetlana Schmidt, Ingrid E. Scheffer, Alexandra Fischer, Simone C. Yendle, Thierry Bienvenu, Juliette Nectoux, Carolyn J. Ellaway, Artur Darmanian, XingZhang Tong, Desiree Cloosterman, Bruce Bennetts, Veena Kalra, Tod Fullston, Jozef Gecz, Timothy C. Cox, John Christodoulou
-
- Journal:
- Twin Research and Human Genetics / Volume 13 / Issue 2 / 01 April 2010
- Published online by Cambridge University Press:
- 21 February 2012, pp. 168-178
-
- Article
-
- You have access Access
- Export citation
-
Rett syndrome (RTT) is a severe neurodevelopmental disorder affecting females almost exclusively and is characterized by a wide spectrum of clinical manifestations. Mutations in the X-linked methyl-CpG-binding protein 2 (MECP2) gene have been found in up to 95% of classical RTT cases and a lesser proportion of atypical cases. Recently, mutations in another X-linked gene, CDKL5 (cyclin-dependent kinase-like 5) have been found to cause atypical RTT, in particular the early onset seizure (Hanefeld variant) and one female with autism. In this study we screened several cohorts of children for CDKL5 mutations, totaling 316 patients, including individuals with a clinical diagnosis of RTT but who were negative for MECP2 mutations (n = 102), males with X-linked mental retardation (n = 9), patients with West syndrome (n = 52), patients with autism (n = 59), patients with epileptic encephalopathy (n = 33), patients with Aicardi syndrome (n = 7) and other patients with intellectual disability with or without seizures (n = 54). In all, seven polymorphic variations and four de novo mutations (c.586C>T [p.S196L]; c.58G>C [p.G20R]; c.2504delC [p.P835fs]; deletion of exons 1 - 3) were identified, and in all instances of the latter the clinical phenotype was that of an epileptic encephalopathy. These results suggest that pathogenic CDKL5 mutations are unlikely to be identified in the absence of severe early-onset seizures and highlight the importance of screening for large intragenic and whole gene deletions.
![](/core/cambridge-core/public/images/lazy-loader.gif)