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Deficiency of the Zinc Finger Protein ZFP106 Causes Motor and Sensory
Human Molecular Genetics, 2016, Vol. 25, No. 2 291–307 doi: 10.1093/hmg/ddv471 Advance Access Publication Date: 24 November 2015 Original Article ORIGINAL ARTICLE Deficiency of the zinc finger protein ZFP106 causes Downloaded from https://academic.oup.com/hmg/article/25/2/291/2384594 by guest on 23 September 2021 motor and sensory neurodegeneration Peter I. Joyce1, Pietro Fratta2,†, Allison S. Landman1,†, Philip Mcgoldrick2,†, Henning Wackerhage3, Michael Groves2, Bharani Shiva Busam3, Jorge Galino4, Silvia Corrochano1, Olga A. Beskina2, Christopher Esapa1, Edward Ryder4, Sarah Carter1, Michelle Stewart1, Gemma Codner1, Helen Hilton1, Lydia Teboul1, Jennifer Tucker1, Arimantas Lionikas3, Jeanne Estabel5, Ramiro Ramirez-Solis5, Jacqueline K. White5, Sebastian Brandner2, Vincent Plagnol6, David L. H. Bennet4,AndreyY.Abramov2,LindaGreensmith2,*, Elizabeth M. C. Fisher2,* and Abraham Acevedo-Arozena1,* 1MRC Mammalian Genetics Unit, Harwell, Oxfordshire OX11 0RD, UK, 2UCL Institute of Neurology and MRC Centre for Neuromuscular Disease, Queen Square, London WC1N 3BG, UK, 3Health Sciences, University of Aberdeen, Aberdeen AB25 2ZD, UK, 4Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford OX3 9DU, UK, 5Sanger Institute, Wellcome Trust Genome Campus, Hinxton, Cambridgeshire CB10 1SA, UK and 6UCL Genetics Institute, London WC1E 6BT, UK *To whom correspondence should be addressed. Email: [email protected] (A.A.)/e.fi[email protected] (E.M.C.F.)/[email protected] (L.G.) Abstract Zinc finger motifs are distributed amongst many eukaryotic protein families, directing nucleic acid–protein and protein–protein interactions. Zinc finger protein 106 (ZFP106) has previously been associated with roles in immune response, muscle differentiation, testes development and DNA damage, although little is known about its specific function. -
The Materials and Components for Missiles Innovation and Technology Partnership, MCM ITP Is a Dstl and DGA Sponsored Research Fu
The Materials and Components for Missiles Innovation and Technology Partnership, MCM ITP is a dstl and DGA sponsored research fund open to all UK or French companies and academic institutions. Launched in 2007, the MCM ITP develops novel, exploitable technologies for generation-after-next missile systems. The MCM ITP aims to consolidate the UK-French Complex Weapons capability, strengthen the technological base and allow better understanding of common future needs. The programme manages a portfolio of over 100 cutting-edge technologies which hold the promise of major advances, but are still at the laboratory stage today. The MCM ITP is aligned into eight technical domains, each of which is led by one of the MCM ITP industrial consortium partners1. 1 The MCM ITP Industrial Consortium partners are: MBDA; THALES; Roxel; Selex ES; Safran Microturbo; QinetiQ; Nexter Munitions. Funding The programme is funded equally by the governments and the industrial partners and is composed of research projects on innovative and exploratory technologies and techniques for future missiles. There is strong participation from SMEs and academia with 76 participating in the programme to date, and a total of 121 organisations involved in the overall programme. With an annual budget of up to 12.5M€ and 30% of the budget targeted towards SMEs and Academia, the MCM has become the cornerstone of future collaborative research and technology demonstration programmes for UK-French missile systems. Conference On 21st and 22nd October 2015, DGA, dstl, MBDA and its partners will review the last two years of the MCM ITP programme, and present the technical advances that have been made possible thanks to this cooperative programme. -
Eurofighter World Editorial 2016 • Eurofighter World 3
PROGRAMME NEWS & FEATURES DECEMBER 2016 GROSSETO EXCLUSIVE BALTIC AIR POLICING A CHANGING AIR FORCE FIT FOR THE FUTURE 2 2016 • EUROFIGHTER WORLD EDITORIAL 2016 • EUROFIGHTER WORLD 3 CONTENTS EUROFIGHTER WORLD PROGRAMME NEWS & FEATURES DECEMBER 2016 05 Editorial 24 Baltic policing role 42 Dardo 03 Welcome from Volker Paltzo, Germany took over NATO’s Journalist David Cenciotti was lucky enough to CEO of Eurofighter Jagdflugzeug GmbH. Baltic Air Policing (BAP) mis - get a back seat ride during an Italian Air Force sion in September with five training mission. Read his eye-opening first hand Eurofighters from the Tactical account of what life onboard the Eurofighter Title: Eurofighter Typoon with 06 At the heart of the mix Air Wing 74 in Neuburg, Typhoon is really like. P3E weapons fit. With the UK RAF evolving to meet new demands we speak to Bavaria deployed to Estonia. Typhoon Force Commander Air Commodore Ian Duguid about the Picture: Jamie Hunter changing shape of the Air Force and what it means for Typhoon. 26 Meet Sina Hinteregger By day Austrian Sina Hinteregger is an aircraft mechanic working on Typhoon, outside work she is one of the country’s best Eurofighter World is published by triathletes. We spoke to her Eurofighter Jagdflugzeug GmbH about her twin passions. 46 Power base PR & Communications Am Söldnermoos 17, 85399 Hallbergmoos Find out how Eurofighter Typhoon wowed the Tel: +49 (0) 811-80 1587 crowds at AIRPOWER16, Austria’s biggest Air [email protected] 12 Master of QRA Show. Editorial Team Discover why Eurofighter Typhoon’s outstanding performance and 28 Flying visit: GROSSETO Theodor Benien ability make it the perfect aircraft for Quick Reaction Alert. -
Abstracts of Papers Presented at the Tenth Mammalian Genetics And
Genet. Res., Camb. (2000), 76, pp. 199–214. Printed in the United Kingdom # 2000 Cambridge University Press 199 Abstracts of papers presented at the tenth Mammalian Genetics and Deelopment Workshop (Incorporating the Promega Young Geneticists’ Meeting) held at the Institute of Child Health, Uniersity College London on 17–19Noember 1999 " # Edited by: ANDREW J. COPP AND ELIZABETH M. C. FISHER 1Institute of Child Health, Uniersity College London, 30 Guilford Street, London WC1N 1EH, UK # Neurogenetics Unit, Imperial College School of Medicine, St Mary’s Hospital, Norfolk Place, London W2 1PG, UK Sponsored by: The Genetical Society, Promega (UK) Ltd and B&K Universal Group Ltd Evidence for imprinting in type 2 diabetes: detection of Identification of a locus for primary ciliary dyskinesia parent-of-origin effects at the insulin gene on chromosome 19 " " " STEWART HUXTABLE, PHILIP SAKER, LEMA S. L. SPIDEN , M. MEEKS , A. J. WALNE ,H. # # HADDAD, ANDREW HATTERSLEY, MARK BLAU , H. MUSSAFFI-GEORGY ,H. $ % % WALKER and MARK McCARTHY SIMPSON , M. EL FEHAID , M. CHEEBAB ,M. % % Section of Endocrinology, Imperial College School of AL-DABBAGH , H. D. HAMMUM ,R.M. " " Medicine, St Mary’s Hospital, London, UK GARDINER , E. M. K. CHUNG and H. M. " MITCHISON " Variation at the insulin gene (INS) VNTR is impli- Department of Paediatrics, Royal Free and Uniersity cated in type 1 diabetes, polycystic ovarian syndrome College Medical School, Uniersity College London, # and birthweight. Case-control studies inconsistently UK; Schneider Children’s Medical Center of Israel, $ show class III VNTR association with type 2 diabetes, Petech Tika, Israel; School of Medical Sciences, but may result from population stratification. -
Ew Test and Evaluation - Assuring Survivability and Operational Effectiveness
EW TEST AND EVALUATION - ASSURING SURVIVABILITY AND OPERATIONAL EFFECTIVENESS Dr. Mike Pywell - EW Technologist & Project/Engineering Manager – Typhoon EW Rig Support Equipment Mitch Midgley-Davies – Technical Lead - Typhoon Avionics Sensors Future Capability (Radar/EW) Electromagnetic Engineering Department BAE SYSTEMS, Military Air & Information © BAE Systems 2013. All rights reserved. DEAL 5868 1 Introduction Contents • Survival – man-made and unintentional threats • EW importance to survivability, mission success and affordability • Description of EW systems • Challenges facing the EW Test and Evaluation community • EW T&E process and capabilities • Description of significant developments to date • Moving EW T&E from flight towards modelling and simulation Security statement: Unclassified © BAE Systems 2013. All rights reserved. DEAL 5868 2 Survival - Unintentional Threats • Lightning Strike • High intensity radiated fields (HiRF) • Electro-static discharge Bruce Fisher’s F-106B Typhoon undergoing full-threat Delta Dart was struck 714 lightning strike testing in BAE times during lightning Lightning strikes near taxying C-130 SYSTEMS EW Test Facility Hercules in Iraq research missions USAF Photo: Senior Airman James Croxon NASA Report SP-2003-4529 © BAE Systems 2013. All rights reserved. DEAL 5868 3 Survival - Man-made Threats © Reproduced with the kind permission of Jane’s Information Group © BAE Systems 2013. All rights reserved. DEAL 5868 4 Typical Missile Engagement Ranges vs. Missile Volume SA-21 SA-20 SA-10 SA-12 • Patriot SA-11, SA-17 SA-6 SA-3 SA-15 SA-19 SA-8 SA-16 SA-13 Roland SA-9 •SA-18 SA-14 • Rapier • Javelin SA-7 (USAF Photo by Airman 1st Class Jonathan Snyder) © BAE Systems 2013. -
Air Connect UK Report
National Sector Information - UK The U.K. has both civil and military aerospace industry and an important space sector. Current employment numbers are: 109,000 direct employees and 120,000 indirect jobs (ie in the supply chain). By value this is 50% civilian aerospace and 50% military aerospace. OEMs Rolls-Royce Airbus BAE Systems Bombardier Agusta Westland MBDA Important Suppliers GKN Selex ES Thales GE UTAS Raytheon Spirit Aerosystems Eaton Aerospace Martin Baker Marshalls Honeywell Safran Locations The industry is present in every region of the U.K. but the most concentrated employee numbers are in the south west, north west, East Midlands and Scotland. 75% of the revenues of the industry are from export sales. On the civil side, much of this is attributable to sales of Airbus aircraft and Rolls-Royce engines. Also important are Bombardier, with wings for the new C Series produced in Belfast, and Agusta Westland in the rotary market. In the military field, exports are dominated by Hawk aircraft, but significant orders have been won for Typhoon. BAE Systems are building rear empennage structures for all F-35 aircraft worldwide. The U.K. Supply chain supports those OEMs above and are also significant suppliers for Boeing, Lockheed Martin, Northrop Grumman and other USA companies. Top 5 companies by employment numbers: BAE Systems 34,800 employees Rolls-Royce - 24,000 employees Airbus - 15,000 employees Bombardier - 6000 employees GKN - 3000 employees Economic indicators In 2013, the government listed 634 enterprises as being in the aerospace industry, and total turnover was £24.7 billion in that year. -
Leonardo Finmeccanica
Integrated Border Control Security Solution 09 November 2016 Topics . Leonardo – a new beginning . Leonardo Integrated Border Control Security Solution . Focus on Video Analysis capabilities and technologies . References 2 © Leonardo - Finmeccanica - Società per azioni 2016: A new beginning One Company, Stronger Together 1 January 2016: the One Company is born through merger operations for the incorporation of OTO Melara and WASS into Finmeccanica and the absorption of the activities carried out by AgustaWestland, Alenia Aermacchi and Selex ES. The industrial entity maintains Parent Company and Corporate Centre functions for DRS Technologies, MBDA, Telespazio, Thales Alenia Space, and ATR. Finmeccanica is now Leonardo 28 April 2016: our name changes to Leonardo, inspired by Leonardo da Vinci, a universally recognised symbol of creativity and innovation. Leonardo represents the ideal bridge between historical legacy and our future in the high- tech industrial sectors. 3 © Leonardo - Finmeccanica - Società per azioni Our Business Leonardo is a global high-tech company and one of the key actors in Aerospace, Defence and Security worldwide. Helicopters . Aircraft SUBSIDIARIES AND JOINT VENTURES . Aerostructures . DRS Technologies (100% Leonardo) . Telespazio (67% Leonardo and 33% Thales) DIVISIONSHELICOPTERS SPACE . Airborne & Space Systems . Thales Alenia Space (67% Thales and 33% Leonardo) . MBDA (37.5% BAE Systems, 37.5% Airbus Group, 25% Leonardo) . Land & Naval Defence Electronics . ATR (50% Leonardo and 50% Airbus Group) . Defence Systems -
(LSSC) Consortium Signal Processing Solutions for the Networked Battlespace
University Defence Research Collaboration (UDRC) Signal Processing in a Networked Battlespace Loughborough, Surrey, Strathclyde and Cardiff (LSSC) Consortium Signal Processing Solutions for the Networked Battlespace Director: Jonathon Chambers FREng CEng FIET FIEEE Sensor Signal Processing for Defence: Strand Palace Hotel, Wednesday 4th December 2013 University Defence Research Collaboration (UDRC) Signal Processing in a Networked Battlespace Academic Team Director (CD) Deputy Director (CDD) Additional Staff Work Package Leaders Professors John McWhirter FRS FREng and Ian Proudler have over 50 years of defence signal processing experience at RSRE, DERA and QinetiQ University Defence Research Collaboration (UDRC) Signal Processing in a Networked Battlespace Research Associates Team Fran Ioannis Anastasia Carmine Cemre Miao Swati Keith Seven PhD students supported by LSSC universities started in October 2013 University Defence Research Collaboration (UDRC) Signal Processing in a Networked Battlespace Industrial Supporters QinetiQ Ltd., Malvern Prof. M. Macleod Selex ES, Edinburgh Dr. A. Colquhoun Thales UK, Reading Prof. C. Firth Texas Instruments, Europe Dr. I. Hunter PrismTech Group Ltd., Stirling Dr. K. Steele Mathworks, Glasgow Dr. J. Bowman PDRAs and PhDs will spend secondments with these companies to extend the scope of data set generation, algorithm evaluation and real- time realization. University Defence Research Collaboration (UDRC) Signal Processing in a Networked Battlespace Research Office Team Arif Syed Jeanette Guida To maximise -
The Future of the Defence Supply Chain
Discounts: 1 star officers and above go for free Venue: The Portman Hotel (Radisson Blu), London Main conference: 18th-19th February THE FUTURE OF THE DEFENCE SUPPLY CHAIN 1. Presentations from the Eurofighter program, Rolls Royce Defence and Boeing Defence UK on the challenges they face in the life cycle management of their specific platforms. A unique opportunity to hear about the management of such large and complex supply chains and how you can apply their solutions to your operations 2. Hear from representatives of AFRICOM, the Kenyan Defence Force and the Spanish Army concerning their operations in Africa and how they will be working with the private sector in the future to support their operations in the region 3. Attend a dedicated segment, examining the transformation of logistical support from the perspective of several key militaries including the UK MoD and the Logistics Commodities Services Transformation or the Polish Army – learn about future business opportunities and how their experience can be applied to your supply chains 4. Take part in a champagne roundtable discussion covering topics such as product life cycle management, performance-based logistics and a dedicated session covering the regional challenges of operating in Africa 5. Engage in panel sessions plotting the future of IT Support for both military logistics and the life cycle management and supply chains of defence manufacturers – how can these solutions make your job easier? MAJOR DEFENCE COMPANIES PRESENTING AT DEFENCE LOGISTICS 2015: Senior international speaker panel includes: Major General John Major General Ramon Major General Samuel Brigadier General Captain Rod J. Broadmeadow, Pardo De Santayana W. -
Radar & EW in The
Radar & EW in the RAF Professor Steve Roberts VP Strategy, Airborne & Space Systems Division Visiting Professor in EW Systems Cranfield University & UK Defence Academy Introduction Electro-Magnetic Operations : • Determine enemy use of Electromagnetic Spectrum • Degrade or prevent enemy use of Electromagnetic Spectrum • Maintain friendly ability to use Electromagnetic Spectrum Alphabet Soup: • EC, EW, IW, NAVWAR, Cyber • AI, AESA, RDF, Radar • ESM, RCM, ECM, ECCM, EPM, ELINT, COMINT, SIGINT ES, ED, EA are the new terms • Electronic Surveillance : Collecting information from the electromagnetic spectrum to understand the opponent’s capabilities and actions: • Electronic Defence : Protecting friendly forces from threats that use the electromagnetic spectrum by providing warning of activity and taking action to degrade or prevent an attack • Electronic Attack : Deliberate actions to attack opponents through the electromagnetic spectrum in order to degrade or destroy their capability Marconi and Ferranti were involved from the beginning – both now Selex ES The Electromagnetic Spectrum 1914-18 “Y” Stations in World War 1 “Y” Stations in UK and France networked by telephone and telegraph to Whitehall Marconi “C” Valve Enabled high sensitivity receiver Marconi Bellini-Tosi DF Receiver 1935 – Watson Watt experiments These experiments were of a bistatic radar configuration Daventry – BBC Radio Chain Home Chain Home RF: 20-50MHz PRF: 25Hz PW: 20µs Battle of Britain (1940) and The Blitz (1940-41) Radio Countermeasures 1939-45 Support to the British -
EWCI Final Annoucement 2016
BHARAT ELECTRONICS Fourth International Conference on Electronic Warfare EWCI 2016 22 to 25 February 2016 EW : Partner for Self-Reliance Venue : National Science Seminar Complex Indian Institute of Science Bangalore The Fourth EW International Conference India (EWCI 2016) The Fourth International Conference on Electronic Warfare (EWCI 2016) is the latest event in the benchmark EWCI Conference Series in the advanced field of EW. The Conference is being organised by the award winning India Chapter of Association of Old Crows (AOC), Bangalore. The Conference is supported by Defence Research and Development Organisation (DRDO), India. Bharat Electronics Limited (BEL), Bangalore is the patronising sponsor of the event. The Conference addresses the Technical and Commercial Needs of Operational Users, Planners, Developers, Procurers, Testers and Trainers of the latest EW Technologies and Systems. A large scale Indoor Exhibition will accompany the Conference, displaying the latest EW products from International EW Organisations. There will be an intense one-day Pre-Conference Tutorials preceding the Conference. The Conference is envisaged as the important platform for EW Professionals who would share the Research and Development output in the field of EW at the global level. Also keeping in view of the Government of India's “Make in India” policy, the Participants of the Conference will be exposed to the state-of-the-art self reliance activities in the field of EW in India and hence, the theme of the Conference is chosen as “EW: Partner for Self- Reliance”.Final Announcement regarding the Details of the Conference is issued through this Brochure. Technical Papers Presentation The Conference receives a huge response in terms of technical papers internationally, covering all aspects of exploiting Electro Magnetic Spectrum for Electronic Warfare and Information Operations. -
The Social Structure, Ecology and Pathogens of Bats in the UK
The Social Structure, Ecology and Pathogens of Bats in the UK Submitted by Thomas Adam August to the University of Exeter as a thesis for the degree of Doctor of Philosophy in Biological Sciences In September 2012 This thesis is available for library use on the understanding that it is copyright material and that no quotation from the thesis may be published without proper acknowledgement. I certify that all material in this thesis which is not my own work has been identified and that no material has previously been submitted and approved for the award of a degree by this or any other university. Signature: ………………………………………………………….. 1 2 Dedicated to the memory of Charles William Stewart Hartley 3 4 Abstract This thesis examines the ecology, parasites and pathogens of three insectivorous bat species in Wytham Woods, Oxfordshire; Myotis nattereri (Natterer’s bat), M. daubentonii (Daubenton’s bat) and Plecotus auritus (Brown long-eared bat). The population structure was assessed by monitoring associations between ringed individuals, utilising recent advances in social network analysis. Populations of both M. daubentonii and M. nattereri were found to subdivide into tight-knit social groups roosting within small areas of a continuous woodland (average minimum roost home range of 0.23km2 and 0.17km2 respectively). If this population structure is a general attribute of these species it may make them more sensitive to small scale habitat change than previously thought and has implications for how diseases may spread through the population. M. daubentonii had a strong preference for roosts close to water, away from woodland edge and in areas with an easterly aspect.