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Probing the Solution Structure of IκB Kinase (IKK) Subunit γ and Its Interaction with Kaposi Sarcoma-associated Herpes Virus Flice-interacting Protein and IKK Subunit β by EPR Spectroscopy.
Claire Bagnéris, Kacper B Rogala, Mehdi Baratchian, Vlad Zamfir, Micha BA Kunze, Selina Dagless, Katharina F Pirker, Mary K Collins, Benjamin A Hall, Tracey E Barrett, Christopher WM Kay
Sep 05, 2018
Viral flice-interacting protein (vFLIP), encoded by the oncogenic Kaposi sarcoma-associated herpes virus (KSHV), constitutively activates the canonical nuclear factor κ-light-chain-enhancer of activated B cells (NF-κB) pathway....
IKKγ NEMO NF-kappa B (NF-κB) electron paramagnetic resonance (EPR) Molecular dynamics molecular modeling Protein Structure signalosome vFLIP Virology Amino Acid Motifs Binding Sites Electron Spin Resonance Spectroscopy Herpesvirus 8 Human Humans I-kappa B Kinase Protein Binding Sarcoma Kaposi Viral Proteins
Bone transport with magnetic intramedullary nails in long bone defects.
BACKGROUND: This study describes the outcomes of internal bone transport with magnetic nails in five cases of traumatic segmental femoral bone defects. METHODS: Five patients with open fracture of the femur and diaphyseal bone...
Modified negative pressure wound therapy as an adjunct to antibiotics in the treatment of orthopaedic infected metalwork.
PURPOSE: Infected orthopaedic metalwork is challenging to treat. Negative pressure wound therapy (NPWT) with irrigation is an emerging therapy for infected wounds as an adjunct to antibiotic therapy. The senior author had...
Human influenza A virus H1N1 in marine mammals in California, 2019.
Magdalena Plancarte, Ganna Kovalenko, Julie Baldassano, Ana L Ramírez, Selina Carrillo, Pádraig J Duignan, Ian Goodfellow, Eric Bortz, Jayeeta Dutta, Harm van Bakel, Lark L Coffey
Mar 30, 2023
Microtubules Deform the Nuclear Membrane and Disrupt Nucleocytoplasmic Transport in Tau-Mediated Frontotemporal Dementia.
Francesco Paonessa, Lewis D Evans, Ravi Solanki, Delphine Larrieu, Selina Wray, John Hardy, Stephen P Jackson, Frederick J Livesey
Feb 05, 2019
The neuronal microtubule-associated protein tau, MAPT, is central to the pathogenesis of many dementias. Autosomal-dominant mutations in MAPT cause inherited frontotemporal dementia (FTD), but the underlying pathogenic...
Human influenza A virus H1N1 in marine mammals in California, 2019.
Magdalena Plancarte, Ganna Kovalenko, Julie Baldassano, Ana L Ramírez, Selina Carrillo, Pádraig J Duignan, Ian Goodfellow, Eric Bortz, Jayeeta Dutta, Harm van Bakel, Lark L Coffey
Apr 17, 2023
From 2011-2018, we conducted surveillance in marine mammals along the California coast for influenza A virus (IAV), frequently detecting anti-influenza antibodies and intermittently detecting IAV. In spring 2019, this pattern...
Animals Seals Earless Humans Influenza A virus California Influenza Human Influenza A virus H1N1 Subtype Caniformia
Published by: PLOS ONE
Mitochondrial ROS control neuronal excitability and cell fate in frontotemporal dementia.
Noemí Esteras, Olga Kopach, Marta Maiolino, Vincenzo Lariccia, Salvatore Amoroso, Seema Qamar, Selina Wray, Dmitri A Rusakov, Morana Jaganjac, Andrey Y Abramov
May 31, 2021
INTRODUCTION: The second most common form of early-onset dementia-frontotemporal dementia (FTD)-is often characterized by the aggregation of the microtubule-associated protein tau. Here we studied the mechanism of tau-induced...
4R tau AMPA receptors MAPT 10+16 NMDA receptors Calcium Signaling frontotemporal dementia Glutamate Induced Pluripotent Stem Cells mitochondrial antioxidants mitochondrial reactive oxygen species Tau frontotemporal dementia Humans Induced Pluripotent Stem Cells Mitochondria Neurons Reactive Oxygen Species Receptors N-Methyl-D-Aspartate tau Proteins
The Parkinson's disease-linked proteins Fbxo7 and Parkin interact to mediate mitophagy.
Victoria S Burchell, David E Nelson, Alvaro Sanchez-Martinez, Marta Delgado-Camprubi, Rachael M Ivatt, Joe H Pogson, Suzanne J Randle, Selina Wray, Patrick A Lewis, Henry Houlden, Andrey Y Abramov, John Hardy, Nicholas W Wood, Alexander J Whitworth, Heike Laman, Helene Plun-Favreau
Aug 08, 2017
Compelling evidence indicates that two autosomal recessive Parkinson's disease genes, PINK1 (PARK6) and Parkin (PARK2), cooperate to mediate the autophagic clearance of damaged mitochondria (mitophagy). Mutations in the F-box...
Animals Animals Genetically Modified Carbonyl Cyanide m-Chlorophenyl Hydrazone Cell Line Tumor Cells Cultured Drosophila F-Box Proteins Female Fertility FIBROBLASTS Humans Male Microtubule-Associated Proteins Mitochondria Mitochondrial Degradation Mutation Parkinson Disease Protein Transport Proton Ionophores RNA Small Interfering Time Factors Ubiquitin-Protein Ligases Ubiquitination
UK Multicenter Prospective Evaluation of the Leibovich Score in Localized Renal Cell Carcinoma
Naveen S Vasudev, Michelle Hutchinson, Sebastian Trainor, Roisean Ferguson, Selina Bhattarai, Adebanji Adeyoju, Jon Cartledge, Michael Kimuli, Shibendra Datta, Damian Hanbury, David Hrouda, Grenville Oades, Poulam Patel, Naeem Soomro, Grant D Stewart, Mark Sullivan, Jeff Webster, Michael Messenger, Peter J Selby, Rosamonde E Banks
Sep 23, 2019
OBJECTIVE: To examine changes in outcome by the Leibovich score using contemporary and historic cohorts of patients presenting with renal cell carcinoma (RCC) PATIENTS AND METHODS: Prospective observational multicenter cohort...
Palaeoproteomic evidence identifies archaic hominins associated with the Châtelperronian at the Grotte du Renne.
Frido Welker, Mateja Hajdinjak, Sahra Talamo, Klervia Jaouen, Michael Dannemann, Francine David, Michèle Julien, Matthias Meyer, Janet Kelso, Ian Barnes, Selina Brace, Pepijn Kamminga, Roman Fischer, Benedikt M Kessler, John R Stewart, Svante Pääbo, Matthew J Collins, Jean-Jacques Hublin
Jul 18, 2019
In Western Europe, the Middle to Upper Paleolithic transition is associated with the disappearance of Neandertals and the spread of anatomically modern humans (AMHs). Current chronological, behavioral, and biological models of...
Châtelperronian Neandertal ZooMS palaeoproteomics Alleles Animals Archaeology Bayes Theorem Bone and Bones Carbon Isotopes Chromatography Liquid Collagen Type X DNA Mitochondrial France Hominidae Humans Paleontology Polymorphism Single Nucleotide Proteomics Radiometric Dating Tandem Mass Spectrometry
Increased circulating levels of Factor H-Related Protein 4 are strongly associated with age-related macular degeneration.
Valentina Cipriani, Laura Lorés-Motta, Fan He, Dina Fathalla, Viranga Tilakaratna, Selina McHarg, Nadhim Bayatti, İlhan E Acar, Carel B Hoyng, Sascha Fauser, Anthony T Moore, John RW Yates, Eiko K de Jong, B Paul Morgan, Anneke I den Hollander, Paul N Bishop, Simon J Clark
Feb 06, 2021
Aged Apolipoproteins Capillaries Case-Control Studies Complement Activation Complement Factor H Genetic Predisposition to Disease Genome-Wide Association Study Haplotypes Humans Intracellular Signaling Peptides and Proteins LIM Domain Proteins Liver Macular degeneration Muscle Proteins Polymorphism Single Nucleotide Retina
Dysregulation at multiple points of the kynurenine pathway is a ubiquitous feature of renal cancer
Nick Hornigold, Karen R Dunn, Rachel A Craven, Alexandre Zougman, Sebastian Trainor, Rebecca Shreeve, Joanne Brown, Helen Sewell, Michael Shires, Margaret Knowles, Tsutomu Fukuwatari, Eamonn R Maher, Julie Burns, Selina Bhattarai, Mini Menon, Alvis Brazma, Ghislaine Scelo, Lara Feulner, Yasser Riazalhosseini, Mark Lathrop, Adrian Harris, Peter J Selby, Rosamonde E Banks, Naveen S Vasudev
May 22, 2020
BACKGROUND: Indoleamine 2,3-dioxygenase (IDO), the first step in the kynurenine pathway (KP), is upregulated in some cancers and represents an attractive therapeutic target given its role in tumour immune evasion. However, the...
Mouse screen reveals multiple new genes underlying mouse and human hearing loss.
Neil J Ingham, Selina A Pearson, Valerie E Vancollie, Victoria Rook, Morag A Lewis, Jing Chen, Annalisa Buniello, Elisa Martelletti, Lorenzo Preite, Chi Chung Lam, Felix D Weiss, Zӧe Powis, Pim Suwannarat, Christopher J Lelliott, Sally J Dawson, Jacqueline K White, Karen P Steel
Feb 04, 2021
Adult-onset hearing loss is very common, but we know little about the underlying molecular pathogenesis impeding the development of therapies. We took a genetic approach to identify new molecules involved in hearing loss by...
Targeting of Slc25a21 is associated with orofacial defects and otitis media due to disrupted expression of a neighbouring gene.
Simon Maguire, Jeanne Estabel, Neil Ingham, Selina Pearson, Edward Ryder, Damian M Carragher, Nicolas Walker, Sanger MGP Slc25a21 Project Team, James Bussell, Wai-In Chan, Thomas M Keane, David J Adams, Cheryl L Scudamore, Christopher J Lelliott, Ramiro Ramírez-Solis, Natasha A Karp, Karen P Steel, Jacqueline K White, Anna-Karin Gerdin
Feb 18, 2021
Homozygosity for Slc25a21(tm1a(KOMP)Wtsi) results in mice exhibiting orofacial abnormalities, alterations in carpal and rugae structures, hearing impairment and inflammation in the middle ear. In humans it has been hypothesised...
Alleles Animals Dicarboxylic Acid Transporters Exons Female Gene Expression Regulation Genetic Engineering Homozygote Humans Male Membrane Transport Proteins Mice Mice Knockout Mitochondrial Membrane Transport Proteins Mouth Abnormalities Mutation Otitis Media PAX9 Transcription Factor Paired Box Transcription Factors Signal Transduction
Dysregulation at multiple points of the kynurenine pathway is a ubiquitous feature of renal cancer
Nick Hornigold, Karen R. Dunn, Rachel A. Craven, Alexandre Zougman, Sebastian Trainor, Rebecca Shreeve, Joanne Brown, Helen Sewell, Michael Shires, Margaret Knowles, Tsutomu Fukuwatari, Eamonn R. Maher, Julie Burns, Selina Bhattarai, Mini Menon, Alvis Brazma, Ghislaine Scelo, Lara Feulner, Yasser Riazalhosseini, Mark Lathrop, Adrian Harris, Peter J. Selby, Rosamonde E. Banks, Naveen S. Vasudev
May 11, 2021
Progressive Motor Neuron Pathology and the Role of Astrocytes in a Human Stem Cell Model of VCP-Related ALS.
Claire E Hall, Zhi Yao, Minee Choi, Giulia E Tyzack, Andrea Serio, Raphaelle Luisier, Jasmine Harley, Elisavet Preza, Charlie Arber, Sarah J Crisp, P Marc D Watson, Dimitri M Kullmann, Andrey Y Abramov, Selina Wray, Russell Burley, Samantha HY Loh, L Miguel Martins, Molly M Stevens, Nicholas M Luscombe, Christopher R Sibley, Andras Lakatos, Jernej Ule, Sonia Gandhi, Rickie Patani
Nov 29, 2018
Motor neurons (MNs) and astrocytes (ACs) are implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS), but their interaction and the sequence of molecular events leading to MN death remain unresolved. Here, we...
amyotrophic lateral sclerosis (ALS) astrocytes (ACs) Disease Modeling induced pluripotent stem cells (iPSCs) motor neurons (MNs) Amyotrophic Lateral Sclerosis Astrocytes Cell Survival DNA-Binding Proteins Endoplasmic Reticulum Endoplasmic reticulum stress Humans Induced Pluripotent Stem Cells Membrane potential Mitochondrial Mitochondria Models Biological Motor Neurons Mutation Nerve Degeneration Neurogenesis Oxidative Stress Phenotype Synapses Valosin Containing Protein
The ProtecT trial
Richard J Bryant, Jon Oxley, Grace J Young, Janet A Lane, Chris Metcalfe, Michael Davis, Emma L Turner, Richard M Martin, John R Goepel, Murali Varma, David F Griffiths, Ken Grigor, Nick Mayer, Anne Y Warren, Selina Bhattarai, John Dormer, Malcolm Mason, John Staffurth, Eleanor Walsh, Derek J Rosario, James WF Catto, David E Neal, Jenny L Donovan, Freddie C Hamdy, ProtecT study group
May 19, 2020
OBJECTIVE: To test the hypothesis that the baseline clinico-pathological features of the men with localized prostate cancer (PCa) included in the ProtecT (Prostate Testing for Cancer and Treatment) trial who progressed (n = 198)...
The ARCA Registry
Andreas Traschütz, Selina Reich, Astrid D. Adarmes, Mathieu Anheim, Mahmoud Reza Ashrafi, Jonathan Baets, A. Nazli Basak, Enrico Bertini, Bernard Brais, Cynthia Gagnon, Janina Gburek-Augustat, Hasmet A. Hanagasi, Anna Heinzmann, Rita Horvath, Peter De Jonghe, Christoph Kamm, Peter Klivenyi, Thomas Klopstock, Martina Minnerop, Alexander Münchau, Mathilde Renaud, Richard H. Roxburgh, Filippo M. Santorelli, Tommaso Schirinzi, Deborah A. Sival, Dagmar Timmann, Stefan Vielhaber, Michael Wallner, Bart P. van de Warrenburg, Ginevra Zanni, Stephan Zuchner, Thomas Klockgether, Rebecca Schüle, Ludger Schöls, PREPARE Consortium, Matthis Synofzik
Jul 28, 2021
Autosomal recessive cerebellar ataxias (ARCAs) form an ultrarare yet expanding group of neurodegenerative multisystemic diseases affecting the cerebellum and other neurological or non-neurological systems. With the advent of...
Worldwide surveillance of self-reported sitting time
M. Mclaughlin, A. J. Atkin, L. Starr, A. Hall, L. Wolfenden, R. Sutherland, J. Wiggers, A. Ramirez, P. Hallal, M. Pratt, B. M. Lynch, K. Wijndaele, Saiful Adli, Paul A. Gardiner, Ciaran B. Doyle, Angela Meadows, Ruth M. Mabry, Alberto Florez Pregonero, Kabir P. Sadarangani, Nyssa T. Hadgraft, Terry Boyle, Nicolas Aguilar Farias, Jacqueline L. Mair, Siosaia F. Hafoka, Gregore Iven Mielke, Selina Khoo Phaik Lin, Vienna R. McLeod, Chathuranga Ranasinghe, Paul C. Storning, Ing-Mari Dohrn, Falk Müller-Riemenschnieder, Lyutha Al Subhi, Anne Chu Hin Yee, Mayuri Gad, Adilson Marques, Elli Kontostoli
Sep 03, 2020
Abstract: Background: Prolonged sitting time is a risk factor for chronic disease, yet recent global surveillance is not well described. The aims were to clarify: (i) the countries that have collected country-level data on...
Fulminant corticobasal degeneration
Helen Ling, Ellen Gelpi, Karen Davey, Zane Jaunmuktane, Kin Y Mok, Edwin Jabbari, Roberto Simone, Lea R'Bibo, Sebastian Brandner, Matthew J Ellis, Johannes Attems, David Mann, Glenda M Halliday, S Al-Sarraj, J Hedreen, James W Ironside, Gabor G Kovacs, E Kovari, S Love, Jean Paul G Vonsattel, Kieren SJ Allinson, Daniela Hansen, Teisha Bradshaw, Núria Setó-Salvia, Selina Wray, Rohan de Silva, Huw R Morris, Thomas T Warner, John Hardy, Janice L Holton, Tamas Revesz
Jan 15, 2021
Corticobasal degeneration typically progresses gradually over 5-7 years from onset till death. Fulminant corticobasal degeneration cases with a rapidly progressive course were rarely reported (RP-CBD). This study aimed to...
A large scale hearing loss screen reveals an extensive unexplored genetic landscape for auditory dysfunction.
Michael R Bowl, Michelle M Simon, Neil J Ingham, Simon Greenaway, Luis Santos, Heather Cater, Sarah Taylor, Jeremy Mason, Natalja Kurbatova, Selina Pearson, Lynette R Bower, Dave A Clary, Hamid Meziane, Patrick Reilly, Osamu Minowa, Lois Kelsey, International Mouse Phenotyping Consortium, Glauco P Tocchini-Valentini, Xiang Gao, Allan Bradley, William C Skarnes, Mark Moore, Arthur L Beaudet, Monica J Justice, John Seavitt, Mary E Dickinson, Wolfgang Wurst, Martin Hrabe de Angelis, Yann Herault, Shigeharu Wakana, Lauryl MJ Nutter, Ann M Flenniken, Colin McKerlie, Stephen A Murray, Karen L Svenson, Robert E Braun, David B West, KC Kent Lloyd, David J Adams, Jacqui White, Natasha Karp, Paul Flicek, Damian Smedley, Terrence F Meehan, Helen E Parkinson, Lydia M Teboul, Sara Wells, Karen P Steel, Ann-Marie Mallon, Steve DM Brown
Oct 14, 2020
The developmental and physiological complexity of the auditory system is likely reflected in the underlying set of genes involved in auditory function. In humans, over 150 non-syndromic loci have been identified, and there are...
Fulminant corticobasal degeneration
Helen Ling, Ellen Gelpi, Karen Davey, Zane Jaunmuktane, Kin Y. Mok, Edwin Jabbari, Roberto Simone, Lea R’Bibo, Sebastian Brandner, Matthew J. Ellis, Johannes Attems, David Mann, Glenda M. Halliday, S. Al-Sarraj, J. Hedreen, James W. Ironside, Gabor G. Kovacs, E. Kovari, S. Love, Jean Paul G. Vonsattel, Kieren S. J. Allinson, Daniela Hansen, Teisha Bradshaw, Núria Setó-Salvia, Selina Wray, Rohan de Silva, Huw R. Morris, Thomas T. Warner, John Hardy, Janice L. Holton, Tamas Revesz
Jun 06, 2020
Abstract: Corticobasal degeneration typically progresses gradually over 5–7 years from onset till death. Fulminant corticobasal degeneration cases with a rapidly progressive course were rarely reported (RP-CBD). This study aimed...
Clinico-Genetic, Imaging and Molecular Delineation of COQ8A-Ataxia
Andreas Traschütz, Tommaso Schirinzi, Lucia Laugwitz, Nathan H Murray, Craig A Bingman, Selina Reich, Jan Kern, Anna Heinzmann, Gessica Vasco, Enrico Bertini, Ginevra Zanni, Alexandra Durr, Stefania Magri, Franco Taroni, Alessandro Malandrini, Jonathan Baets, Peter De Jonghe, Willem de Ridder, Matthieu Bereau, Stephanie Demuth, Christos Ganos, A Nazli Basak, Hasmet Hanagasi, Semra Hiz Kurul, Benjamin Bender, Ludger Schöls, Ute Grasshoff, Thomas Klopstock, Rita Horvath, Bart van de Warrenburg, Lydie Burglen, Christelle Rougeot, Claire Ewenczyk, Michel Koenig, Filippo M Santorelli, Mathieu Anheim, Renato P Munhoz, Tobias Haack, Felix Distelmaier, David J Pagliarini, Hélène Puccio, Matthis Synofzik
May 18, 2020
OBJECTIVE: To foster trial-readiness of coenzyme Q8A (COQ8A)-ataxia, we map the clinicogenetic, molecular, and neuroimaging spectrum of COQ8A-ataxia in a large worldwide cohort, and provide first progression data, including...
The ARCA Registry
Andreas Traschütz, Selina Reich, Astrid D Adarmes, Mathieu Anheim, Mahmoud Reza Ashrafi, Jonathan Baets, A Nazli Basak, Enrico Bertini, Bernard Brais, Cynthia Gagnon, Janina Gburek-Augustat, Hasmet A Hanagasi, Anna Heinzmann, Rita Horvath, Peter De Jonghe, Christoph Kamm, Peter Klivenyi, Thomas Klopstock, Martina Minnerop, Alexander Münchau, Mathilde Renaud, Richard H Roxburgh, Filippo M Santorelli, Tommaso Schirinzi, Deborah A Sival, Dagmar Timmann, Stefan Vielhaber, Michael Wallner, Bart P van de Warrenburg, Ginevra Zanni, Stephan Zuchner, Thomas Klockgether, Rebecca Schüle, Ludger Schöls, PREPARE Consortium, Matthis Synofzik
Aug 17, 2021
Autosomal recessive cerebellar ataxias (ARCAs) form an ultrarare yet expanding group of neurodegenerative multisystemic diseases affecting the cerebellum and other neurological or non-neurological systems. With the advent of...
Worldwide surveillance of self-reported sitting time
M. Mclaughlin, A. J. Atkin, L. Starr, A. Hall, L. Wolfenden, R. Sutherland, J. Wiggers, A. Ramirez, P. Hallal, M. Pratt, B. M. Lynch, K. Wijndaele, Saiful Adli, Paul A. Gardiner, Ciaran B. Doyle, Angela Meadows, Ruth M. Mabry, Alberto Florez Pregonero, Kabir P. Sadarangani, Nyssa T. Hadgraft, Terry Boyle, Nicolas Aguilar Farias, Jacqueline L. Mair, Siosaia F. Hafoka, Gregore Iven Mielke, Selina Khoo Phaik Lin, Vienna R. McLeod, Chathuranga Ranasinghe, Paul C. Storning, Ing-Mari Dohrn, Falk Müller-Riemenschnieder, Lyutha Al Subhi, Anne Chu Hin Yee, Mayuri Gad, Adilson Marques, Elli Kontostoli
Sep 03, 2021
Abstract: Background: Prolonged sitting time is a risk factor for chronic disease, yet recent global surveillance is not well described. The aims were to clarify: (i) the countries that have collected country-level data on...
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