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Wyświetlanie 1-54 z 54
Tytuł :
Comparison between low-level 50 Hz and 900 MHz electromagnetic stimulation on single channel ionic currents and on firing frequency in dorsal root ganglion isolated neurons
Autorzy :
Marchionni, I.
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Źródło :
In BBA - Biomembranes 2006 1758(5):597-605
Czasopismo naukowe
Tytuł :
Mitochondria form contact sites with the nucleus to couple prosurvival retrograde response.
Autorzy :
Desai R; Department of Comparative Biomedical Sciences, The Royal Veterinary College, University of London, Royal College Street, London NW1 0TU, UK.
East DA; Department of Comparative Biomedical Sciences, The Royal Veterinary College, University of London, Royal College Street, London NW1 0TU, UK.
Hardy L; Department of Comparative Biomedical Sciences, The Royal Veterinary College, University of London, Royal College Street, London NW1 0TU, UK.
Faccenda D; Department of Comparative Biomedical Sciences, The Royal Veterinary College, University of London, Royal College Street, London NW1 0TU, UK.
Rigon M; Department of Comparative Biomedical Sciences, The Royal Veterinary College, University of London, Royal College Street, London NW1 0TU, UK.
Crosby J; Department of Comparative Biomedical Sciences, The Royal Veterinary College, University of London, Royal College Street, London NW1 0TU, UK.
Alvarez MS; Department of Comparative Biomedical Sciences, The Royal Veterinary College, University of London, Royal College Street, London NW1 0TU, UK.
Singh A; Department of Comparative Biomedical Sciences, The Royal Veterinary College, University of London, Royal College Street, London NW1 0TU, UK.
Mainenti M; Department of Comparative Biomedical Sciences, The Royal Veterinary College, University of London, Royal College Street, London NW1 0TU, UK.
Hussey LK; Department of Comparative Biomedical Sciences, The Royal Veterinary College, University of London, Royal College Street, London NW1 0TU, UK.
Bentham R; Department of Cell and Developmental Biology, Consortium for Mitochondrial Research (CfMR), University College London, Gower Street, London WC1E 6BT, UK.
Szabadkai G; Department of Cell and Developmental Biology, Consortium for Mitochondrial Research (CfMR), University College London, Gower Street, London WC1E 6BT, UK.; Department of Biomedical Science, University of Padua, Via Ugo Bassi, 35131 Padua, Italy.; Francis Crick Institute, Midland Road, London NW1 AT, UK.
Zappulli V; Department of Comparative Biomedicine and Food Sciences, University of Padua, Viale dell'Universita' 16, 35020 Legnaro (PD), Italy.
Dhoot GK; Department of Comparative Biomedical Sciences, The Royal Veterinary College, University of London, Royal College Street, London NW1 0TU, UK.
Romano LE; William Harvey Research Institute, Barts and the London School of Medicine, Queen Mary University of London, London EC1M 6BQ, UK.
Xia D; Department of Comparative Biomedical Sciences, The Royal Veterinary College, University of London, Royal College Street, London NW1 0TU, UK.
Coppens I; W. Harry Feinstone Department of Molecular Microbiology and Immunology, Johns Hopkins University Baltimore, Baltimore, MD 21205, USA.
Hamacher-Brady A; W. Harry Feinstone Department of Molecular Microbiology and Immunology, Johns Hopkins University Baltimore, Baltimore, MD 21205, USA.
Chapple JP; William Harvey Research Institute, Barts and the London School of Medicine, Queen Mary University of London, London EC1M 6BQ, UK.
Abeti R; Ataxia Centre, Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, Queen Square, London WC1N 3BG, UK.
Fleck RA; Centre for Ultrastructural Imaging, King's College London, London SE1 1UL, UK.
Vizcay-Barrena G; Centre for Ultrastructural Imaging, King's College London, London SE1 1UL, UK.
Smith K; Pathobiology and Population Sciences, The Royal Veterinary College, Hawkshead Lane, North Mymms, Hatfield, Hertfordshire AL9 7TA, UK.
Campanella M; Department of Comparative Biomedical Sciences, The Royal Veterinary College, University of London, Royal College Street, London NW1 0TU, UK. .; Department of Cell and Developmental Biology, Consortium for Mitochondrial Research (CfMR), University College London, Gower Street, London WC1E 6BT, UK.
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Źródło :
Science advances [Sci Adv] 2020 Dec 18; Vol. 6 (51). Date of Electronic Publication: 2020 Dec 18 (Print Publication: 2020).
Typ publikacji :
Journal Article
Czasopismo naukowe
Tytuł :
NH-sulfoximine: A novel pharmacological inhibitor of the mitochondrial F 1 F o -ATPase, which suppresses viability of cancerous cells.
Autorzy :
Strobbe D; Department of Biology, University of Rome 'Tor Vergata', Rome, Italy.
Pecorari R; Department of Biology, University of Rome 'Tor Vergata', Rome, Italy.
Conte O; Department of Biology, University of Rome 'Tor Vergata', Rome, Italy.
Minutolo A; Department of Cell and Developmental Biology, Consortium for Mitochondrial Research (CfMR), University College London, London, UK.
Hendriks CMM; Institute of Organic Chemistry, RWTH Aachen University, Aachen, Germany.
Wiezorek S; Institute of Organic Chemistry, RWTH Aachen University, Aachen, Germany.
Faccenda D; Department of Comparative Biomedical Sciences, The Royal Veterinary College, University of London, London, UK.
Abeti R; Ataxia Centre, Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, Queen Square London, London, WC1N 3BG, UK.
Montesano C; Department of Biology, University of Rome 'Tor Vergata', Rome, Italy.
Bolm C; Institute of Organic Chemistry, RWTH Aachen University, Aachen, Germany.
Campanella M; Department of Comparative Biomedical Sciences, The Royal Veterinary College, University of London, London, UK.; Department of Cell and Developmental Biology, Consortium for Mitochondrial Research (CfMR), University College London, London, UK.; Department of Biology, University of Rome 'Tor Vergata', Rome, Italy.
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Źródło :
British journal of pharmacology [Br J Pharmacol] 2020 Oct 10. Date of Electronic Publication: 2020 Oct 10.
Typ publikacji :
Journal Article
Czasopismo naukowe
Tytuł :
Bi-allelic JAM2 Variants Lead to Early-Onset Recessive Primary Familial Brain Calcification.
Autorzy :
Schottlaender LV; Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, Queen Square, WC1N3BG London, UK; Dubowitz Neuromuscular Centre, UCL Great Ormond Street Institute of Child Health, WC1N 1EH London, UK; Argentine National Scientific and Technological Research Council (CONICET), C1425FQB Buenos Aires, Argentina; FLENI Neurological Research Institute, C1428 AQK Buenos Aires, Argentina.
Abeti R; Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, WC1N3BG London, UK.
Jaunmuktane Z; Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, WC1N3BG London, UK; Division of Neuropathology, The National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, Queen Square, London WC1N 3BG, UK.
Macmillan C; Department of Pediatrics, University of Chicago, Chicago, IL 60637, USA.
Chelban V; Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, Queen Square, WC1N3BG London, UK.
O'Callaghan B; Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, Queen Square, WC1N3BG London, UK.
McKinley J; Department of Neurology, Dublin Neurological Institute at the Mater Misericordiae University Hospital, 57 Eccles St, Dublin 7 DO7W7XF, Ireland; Regional Neurosciences Centre, Royal Victoria Hospital, Belfast BT12 6BA, UK.
Maroofian R; Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, Queen Square, WC1N3BG London, UK.
Efthymiou S; Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, Queen Square, WC1N3BG London, UK.
Athanasiou-Fragkouli A; Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, Queen Square, WC1N3BG London, UK.
Forbes R; Neurology Centre, Southern HSC Trust, Craigavon Area Hospital, Portadown BT63 5QQ, UK.
Soutar MPM; Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, Queen Square, London WC1N 3BG, UK.
Livingston JH; Paediatric Neurology, The Leeds Teaching Hospitals NHS Trust, Leeds General Infirmary, Leeds LS1 3EX, UK.
Kalmar B; Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, Queen Square, WC1N3BG London, UK.
Swayne O; Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, WC1N3BG London, UK; The National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK.
Hotton G; Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, WC1N3BG London, UK; The National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK.
Pittman A; Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, Queen Square, WC1N3BG London, UK.
Mendes de Oliveira JR; Universidade Federal de Pernambuco, Departamento de Neuropsiquiatria, Recife 50670-901, Brazil.
de Grandis M; Aix-Marseille University, Inserm, CNRS, Institut Paoli-Calmettes, CRCM, 13009 Marseille, France.
Richard-Loendt A; Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, Queen Square, London WC1N 3BG, UK.
Launchbury F; Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, Queen Square, London WC1N 3BG, UK.
Althonayan J; Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, WC1N3BG London, UK.
McDonnell G; Regional Neurosciences Centre, Royal Victoria Hospital, Belfast BT12 6BA, UK.
Carr A; Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, Queen Square, WC1N3BG London, UK; The National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK.
Khan S; CENTOGENE AG, Rostock 18055, Germany.
Beetz C; CENTOGENE AG, Rostock 18055, Germany.
Bisgin A; Medical Genetics Department of Medical Faculty & AGENTEM (Adana Genetic Diseases Diagnosis and Treatment Center), Çukurova University, Adana 01330, Turkey.
Tug Bozdogan S; Medical Genetics Department of Medical Faculty & AGENTEM (Adana Genetic Diseases Diagnosis and Treatment Center), Çukurova University, Adana 01330, Turkey.
Begtrup A; GeneDx, 207 Perry Parkway, Gaithersburg, MD 20877, USA.
Torti E; GeneDx, 207 Perry Parkway, Gaithersburg, MD 20877, USA.
Greensmith L; Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, Queen Square, WC1N3BG London, UK.
Giunti P; Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, WC1N3BG London, UK.
Morrison PJ; Centre for Cancer Research and Cell Biology, Queens University, Belfast BT9 7AE, UK.
Brandner S; Division of Neuropathology, The National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, Queen Square, London WC1N 3BG, UK.
Aurrand-Lions M; Aix-Marseille University, Inserm, CNRS, Institut Paoli-Calmettes, CRCM, 13009 Marseille, France.
Houlden H; Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, Queen Square, WC1N3BG London, UK; The National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK; Neurogenetics Laboratory and Clinical Service, The National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK. Electronic address: .
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Corporate Authors :
SYNAPS Study Group; Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, Queen Square, WC1N3BG London, UK.
Źródło :
American journal of human genetics [Am J Hum Genet] 2020 Mar 05; Vol. 106 (3), pp. 412-421.
Typ publikacji :
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms :
Age of Onset*
Alleles*
Genes, Recessive*
Brain Diseases/*genetics
Calcinosis/*genetics
Cell Adhesion Molecules/*genetics
Adolescent ; Adult ; Animals ; Brain Diseases/diagnostic imaging ; Calcinosis/diagnostic imaging ; Child ; Female ; Humans ; Male ; Mice ; Middle Aged ; Pedigree
Czasopismo naukowe
Tytuł :
Xeroderma pigmentosum: overview of pharmacology and novel therapeutic strategies for neurological symptoms.
Autorzy :
Abeti R; Ataxia Centre, Department of Clinical and Movement Neurosciences, University College London, Institute of Neurology London, London, UK.
Zeitlberger A; Ataxia Centre, Department of Clinical and Movement Neurosciences, University College London, Institute of Neurology London, London, UK.
Peelo C; Ataxia Centre, Department of Clinical and Movement Neurosciences, University College London, Institute of Neurology London, London, UK.
Fassihi H; National Xeroderma Pigmentosum Service, St John's Institute of Dermatology Guy's and St Thomas' Foundation Trust, London, UK.
Sarkany RPE; National Xeroderma Pigmentosum Service, St John's Institute of Dermatology Guy's and St Thomas' Foundation Trust, London, UK.
Lehmann AR; Genome Damage and Stability Centre, University of Sussex, Brighton, UK.
Giunti P; Ataxia Centre, Department of Clinical and Movement Neurosciences, University College London, Institute of Neurology London, London, UK.; National Xeroderma Pigmentosum Service, St John's Institute of Dermatology Guy's and St Thomas' Foundation Trust, London, UK.
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Źródło :
British journal of pharmacology [Br J Pharmacol] 2019 Nov; Vol. 176 (22), pp. 4293-4301. Date of Electronic Publication: 2019 Jan 23.
Typ publikacji :
Journal Article; Review
MeSH Terms :
Neurodegenerative Diseases/*drug therapy
Xeroderma Pigmentosum/*drug therapy
Humans ; Neurodegenerative Diseases/etiology ; Neurodegenerative Diseases/genetics ; Neurodegenerative Diseases/metabolism ; Xeroderma Pigmentosum/complications ; Xeroderma Pigmentosum/genetics ; Xeroderma Pigmentosum/metabolism
Czasopismo naukowe
Tytuł :
Complexity of the Genetics and Clinical Presentation of Spinocerebellar Ataxia 17.
Autorzy :
Nethisinghe S; Ataxia Centre, Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, London, United Kingdom.
Lim WN; Ataxia Centre, Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, London, United Kingdom.
Ging H; Ataxia Centre, Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, London, United Kingdom.
Zeitlberger A; Ataxia Centre, Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, London, United Kingdom.
Abeti R; Ataxia Centre, Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, London, United Kingdom.
Pemble S; Neurogenetics Unit, National Hospital for Neurology and Neurosurgery, London, United Kingdom.
Sweeney MG; Neurogenetics Unit, National Hospital for Neurology and Neurosurgery, London, United Kingdom.
Labrum R; Neurogenetics Unit, National Hospital for Neurology and Neurosurgery, London, United Kingdom.
Cervera C; Neurogenetics Unit, National Hospital for Neurology and Neurosurgery, London, United Kingdom.
Houlden H; Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology, London, United Kingdom.; MRC Centre for Neuromuscular Diseases, UCL Queen Square Institute of Neurology, London, United Kingdom.
Rosser E; Department of Clinical Genetics, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom.
Limousin P; Sobell Department of Motor Neuroscience and Movement Disorders, UCL Queen Square Institute of Neurology, London, United Kingdom.
Kennedy A; Chelsea and Westminster Hospital, London, United Kingdom.
Lunn MP; Department of Neuroimmunology, UCL Queen Square Institute of Neurology, London, United Kingdom.
Bhatia KP; Sobell Department of Motor Neuroscience and Movement Disorders, UCL Queen Square Institute of Neurology, London, United Kingdom.
Wood NW; Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, London, United Kingdom.
Hardy J; Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, London, United Kingdom.; The Reta Lila Weston Institute of Neurological Studies, UCL Queen Square Institute of Neurology, London, United Kingdom.
Polke JM; Neurogenetics Unit, National Hospital for Neurology and Neurosurgery, London, United Kingdom.
Veneziano L; Istituto di Farmacologia Traslazionale - National Research Council, Rome, Italy.
Brusco A; Department of Medical Sciences, University of Turin, Turin, Italy.; Medical Genetics Unit, Città della Salute e della Scienza University Hospital, Turin, Italy.
Davis MB; Ataxia Centre, Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, London, United Kingdom.
Giunti P; Ataxia Centre, Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, London, United Kingdom.
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Źródło :
Frontiers in cellular neuroscience [Front Cell Neurosci] 2018 Nov 23; Vol. 12, pp. 429. Date of Electronic Publication: 2018 Nov 23 (Print Publication: 2018).
Typ publikacji :
Journal Article
Czasopismo naukowe
Tytuł :
Calcium Deregulation: Novel Insights to Understand Friedreich's Ataxia Pathophysiology.
Autorzy :
Abeti R; Ataxia Centre, Department of Clinical and Movement Neurosciences, Institute of Neurology, University College London, London, United Kingdom.
Brown AF; Ataxia Centre, Department of Clinical and Movement Neurosciences, Institute of Neurology, University College London, London, United Kingdom.
Maiolino M; Department of Biomedical Sciences and Public Health, School of Medicine, Università Politecnica delle Marche, Ancona, Italy.
Patel S; Department of Cell and Developmental Biology, Division of Biosciences, University College London, London, United Kingdom.
Giunti P; Ataxia Centre, Department of Clinical and Movement Neurosciences, Institute of Neurology, University College London, London, United Kingdom.
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Źródło :
Frontiers in cellular neuroscience [Front Cell Neurosci] 2018 Oct 02; Vol. 12, pp. 264. Date of Electronic Publication: 2018 Oct 02 (Print Publication: 2018).
Typ publikacji :
Journal Article
Czasopismo naukowe
Tytuł :
Novel Nrf2-Inducer Prevents Mitochondrial Defects and Oxidative Stress in Friedreich's Ataxia Models.
Autorzy :
Abeti R; Ataxia Centre, Department of Molecular Neuroscience, UCL Institute of Neurology, London, United Kingdom.
Baccaro A; Ataxia Centre, Department of Molecular Neuroscience, UCL Institute of Neurology, London, United Kingdom.
Esteras N; Department of Molecular Neuroscience, UCL Institute of Neurology, London, United Kingdom.
Giunti P; Ataxia Centre, Department of Molecular Neuroscience, UCL Institute of Neurology, London, United Kingdom.
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Źródło :
Frontiers in cellular neuroscience [Front Cell Neurosci] 2018 Jul 17; Vol. 12, pp. 188. Date of Electronic Publication: 2018 Jul 17 (Print Publication: 2018).
Typ publikacji :
Journal Article
Czasopismo naukowe
Tytuł :
mTOR independent alteration in ULK1 Ser758 phosphorylation following chronic LRRK2 kinase inhibition.
Autorzy :
Manzoni C; School of Pharmacy, University of Reading, Whiteknights, Reading RG6 6AP, U.K. .; Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square, London WC1N 3BG, U.K.
Mamais A; Laboratory of Neurogenetics, National Institute on Aging, National Institutes of Health, Bethesda, MD 20892, U.S.A.
Dihanich S; Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square, London WC1N 3BG, U.K.
Soutar MPM; Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square, London WC1N 3BG, U.K.
Plun-Favreau H; Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square, London WC1N 3BG, U.K.
Bandopadhyay R; Reta Lila Weston Institute of Neurological Studies, UCL Institute of Neurology, 1 Wakefield Street London WC1N 1PJ, U.K.
Abeti R; Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square, London WC1N 3BG, U.K.; Department of Molecular Neuroscience, UCL Institute of Neurology, Ataxia Centre, Queen Square, London WC1N 3BG, United Kingdom.
Giunti P; Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square, London WC1N 3BG, U.K.; Department of Molecular Neuroscience, UCL Institute of Neurology, Ataxia Centre, Queen Square, London WC1N 3BG, United Kingdom.
Hardy J; Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square, London WC1N 3BG, U.K.
R Cookson M; Laboratory of Neurogenetics, National Institute on Aging, National Institutes of Health, Bethesda, MD 20892, U.S.A.
Tooze SA; The Francis Crick Institute, 1 Midland Road, London, NW1 1AT, U.K.
Lewis PA; School of Pharmacy, University of Reading, Whiteknights, Reading RG6 6AP, U.K.; Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square, London WC1N 3BG, U.K.
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Źródło :
Bioscience reports [Biosci Rep] 2018 Apr 20; Vol. 38 (2). Date of Electronic Publication: 2018 Apr 20 (Print Publication: 2018).
Typ publikacji :
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms :
Autophagosomes/*enzymology
Autophagy-Related Protein-1 Homolog/*metabolism
Leucine-Rich Repeat Serine-Threonine Protein Kinase-2/*antagonists & inhibitors
TOR Serine-Threonine Kinases/*metabolism
Animals ; Autophagy-Related Protein-1 Homolog/genetics ; Cell Line ; Leucine-Rich Repeat Serine-Threonine Protein Kinase-2/genetics ; Leucine-Rich Repeat Serine-Threonine Protein Kinase-2/metabolism ; Mechanistic Target of Rapamycin Complex 1/genetics ; Mechanistic Target of Rapamycin Complex 1/metabolism ; Phosphorylation/genetics ; Rats ; Serine/genetics ; Serine/metabolism ; TOR Serine-Threonine Kinases/genetics
Czasopismo naukowe
Tytuł :
Glial cells are functionally impaired in juvenile neuronal ceroid lipofuscinosis and detrimental to neurons.
Autorzy :
Parviainen L; Department of Basic and Clinical Neuroscience, King's College London, Institute of Psychiatry, Psychology & Neuroscience, Maurice Wohl Clinical Neuroscience Institute, 5 Cutcombe Road, London, SE5 9RX, UK.
Dihanich S; Department of Basic and Clinical Neuroscience, King's College London, Institute of Psychiatry, Psychology & Neuroscience, Maurice Wohl Clinical Neuroscience Institute, 5 Cutcombe Road, London, SE5 9RX, UK.
Anderson GW; Department of Basic and Clinical Neuroscience, King's College London, Institute of Psychiatry, Psychology & Neuroscience, Maurice Wohl Clinical Neuroscience Institute, 5 Cutcombe Road, London, SE5 9RX, UK.
Wong AM; Department of Basic and Clinical Neuroscience, King's College London, Institute of Psychiatry, Psychology & Neuroscience, Maurice Wohl Clinical Neuroscience Institute, 5 Cutcombe Road, London, SE5 9RX, UK.
Brooks HR; Department of Basic and Clinical Neuroscience, King's College London, Institute of Psychiatry, Psychology & Neuroscience, Maurice Wohl Clinical Neuroscience Institute, 5 Cutcombe Road, London, SE5 9RX, UK.
Abeti R; Department of Molecular Neuroscience, Institute of Neurology, University College London, Queen's Square, London, WC1N, 3BG, UK.
Rezaie P; Department of Life, Health and Chemical Sciences, The Open University, Walton Hall, Milton Keynes, MK7 6AA, UK.
Lalli G; Wolfson Centre for Age-Related Diseases, King's College London, London, SE1 1UL, UK.
Pope S; Genetics and Genomic Medicine UCL Great Ormond Street Institute of Child Health, University College London, 30 Guilford Street, London, WC1N 1EH, UK.
Heales SJ; Genetics and Genomic Medicine UCL Great Ormond Street Institute of Child Health, University College London, 30 Guilford Street, London, WC1N 1EH, UK.
Mitchison HM; Genetics and Genomic Medicine UCL Great Ormond Street Institute of Child Health, University College London, 30 Guilford Street, London, WC1N 1EH, UK.
Williams BP; Department of Basic and Clinical Neuroscience, King's College London, Institute of Psychiatry, Psychology & Neuroscience, Maurice Wohl Clinical Neuroscience Institute, 5 Cutcombe Road, London, SE5 9RX, UK.
Cooper JD; Department of Basic and Clinical Neuroscience, King's College London, Institute of Psychiatry, Psychology & Neuroscience, Maurice Wohl Clinical Neuroscience Institute, 5 Cutcombe Road, London, SE5 9RX, UK. .; Department of Pediatrics, Harbor-UCLA Medical Center, Los Angeles Biomedical Research Institute and David Geffen School of Medicine UCLA, 1124 West Carson Street, Hanley Hardison Building, Torrance, CA, 90502, USA. .
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Źródło :
Acta neuropathologica communications [Acta Neuropathol Commun] 2017 Oct 17; Vol. 5 (1), pp. 74. Date of Electronic Publication: 2017 Oct 17.
Typ publikacji :
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms :
Brain/*physiopathology
Neuroglia/*physiology
Neuronal Ceroid-Lipofuscinoses/*physiopathology
Neurons/*physiology
Adult ; Aminopeptidases/deficiency ; Aminopeptidases/genetics ; Animals ; Brain/pathology ; Cell Movement/physiology ; Cell Survival/physiology ; Cells, Cultured ; Child ; Coculture Techniques ; Cytoskeleton/metabolism ; Cytoskeleton/pathology ; Dipeptidyl-Peptidases and Tripeptidyl-Peptidases/deficiency ; Dipeptidyl-Peptidases and Tripeptidyl-Peptidases/genetics ; Female ; Glutathione/metabolism ; Humans ; Male ; Membrane Glycoproteins/deficiency ; Membrane Glycoproteins/genetics ; Mice, Inbred C57BL ; Mice, Transgenic ; Molecular Chaperones/genetics ; Neuroglia/pathology ; Neuronal Ceroid-Lipofuscinoses/pathology ; Neurons/pathology ; Serine Proteases/deficiency ; Serine Proteases/genetics ; Young Adult
Czasopismo naukowe
Tytuł :
A reduction in Drp1-mediated fission compromises mitochondrial health in autosomal recessive spastic ataxia of Charlevoix Saguenay.
Autorzy :
Bradshaw TY; William Harvey Research Institute, Barts and the London School of Medicine, Queen Mary University of London, London EC1M 6BQ, United Kingdom .
Romano LE; William Harvey Research Institute, Barts and the London School of Medicine, Queen Mary University of London, London EC1M 6BQ, United Kingdom.
Duncan EJ; William Harvey Research Institute, Barts and the London School of Medicine, Queen Mary University of London, London EC1M 6BQ, United Kingdom.
Nethisinghe S; William Harvey Research Institute, Barts and the London School of Medicine, Queen Mary University of London, London EC1M 6BQ, United Kingdom.
Abeti R; Department of Molecular Neuroscience, UCL Institute of Neurology, London WC1N 3BG, United Kingdom.
Michael GJ; Blizard Institute, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, London E1 2AT, United Kingdom.
Giunti P; Department of Molecular Neuroscience, UCL Institute of Neurology, London WC1N 3BG, United Kingdom.
Vermeer S; Department of Clinical Genetics, The Netherlands Cancer Institute, 1066 CX Amsterdam, The Netherlands.
Chapple JP; William Harvey Research Institute, Barts and the London School of Medicine, Queen Mary University of London, London EC1M 6BQ, United Kingdom .
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Źródło :
Human molecular genetics [Hum Mol Genet] 2016 Aug 01; Vol. 25 (15), pp. 3232-3244. Date of Electronic Publication: 2016 Jun 10.
Typ publikacji :
Journal Article
MeSH Terms :
Mitochondrial Dynamics*
Fibroblasts/*metabolism
GTP Phosphohydrolases/*metabolism
Microtubule-Associated Proteins/*metabolism
Mitochondria/*metabolism
Mitochondrial Membranes/*metabolism
Mitochondrial Proteins/*metabolism
Muscle Spasticity/*metabolism
Spinocerebellar Ataxias/*congenital
Cell Line, Tumor ; Dynamins ; Female ; Fibroblasts/pathology ; GTP Phosphohydrolases/genetics ; Heat-Shock Proteins/genetics ; Heat-Shock Proteins/metabolism ; Humans ; Male ; Microtubule-Associated Proteins/genetics ; Mitochondria/pathology ; Mitochondrial Membranes/pathology ; Mitochondrial Proteins/genetics ; Muscle Spasticity/genetics ; Muscle Spasticity/pathology ; Spinocerebellar Ataxias/genetics ; Spinocerebellar Ataxias/metabolism ; Spinocerebellar Ataxias/pathology
SCR Disease Name :
Spastic ataxia Charlevoix-Saguenay type
Czasopismo naukowe
Tytuł :
Targeting lipid peroxidation and mitochondrial imbalance in Friedreich's ataxia.
Autorzy :
Abeti R; Department of Molecular Neuroscience, UCL, Institute of Neurology, Queen Square, WC1N 3BG London, UK.
Uzun E; Department of Molecular Neuroscience, UCL, Institute of Neurology, Queen Square, WC1N 3BG London, UK.
Renganathan I; Department of Molecular Neuroscience, UCL, Institute of Neurology, Queen Square, WC1N 3BG London, UK.
Honda T; Department of Chemistry, Stony Brook University, Stony Brook, NY 11794, United States.
Pook MA; Ataxia Research Group, Division of Biosciences, Department of Life Sciences, College of Health & Life Sciences Synthetic Biology Theme, Institute of Environment, Health & Societies, Brunel University London, Uxbridge UB8 3PH, UK.
Giunti P; Department of Molecular Neuroscience, UCL, Institute of Neurology, Queen Square, WC1N 3BG London, UK. Electronic address: .
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Źródło :
Pharmacological research [Pharmacol Res] 2015 Sep; Vol. 99, pp. 344-50. Date of Electronic Publication: 2015 Jul 02.
Typ publikacji :
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms :
Friedreich Ataxia/*metabolism
Friedreich Ataxia/*pathology
Lipid Peroxidation/*physiology
Mitochondria/*metabolism
Mitochondria/*pathology
Animals ; Antioxidants/metabolism ; Cell Death/drug effects ; Cells, Cultured ; Disease Models, Animal ; Fibroblasts/drug effects ; Fibroblasts/metabolism ; Friedreich Ataxia/drug therapy ; Iron/metabolism ; Iron-Binding Proteins/metabolism ; Lipid Peroxidation/drug effects ; Membrane Potential, Mitochondrial/drug effects ; Mice ; Mice, Inbred C57BL ; Mitochondria/drug effects ; Oxidative Stress/drug effects ; Phenanthrenes/pharmacology ; Phenotype
Czasopismo naukowe
Tytuł :
Mitochondrial Ca(2+) in neurodegenerative disorders.
Autorzy :
Abeti R; Department of Molecular Neuroscience, UCL Institute of Neurology, London, UK.
Abramov AY; Department of Molecular Neuroscience, UCL Institute of Neurology, London, UK. Electronic address: .
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Źródło :
Pharmacological research [Pharmacol Res] 2015 Sep; Vol. 99, pp. 377-81. Date of Electronic Publication: 2015 May 23.
Typ publikacji :
Journal Article; Review
MeSH Terms :
Calcium/*metabolism
Mitochondria/*metabolism
Neurodegenerative Diseases/*metabolism
Animals ; Cell Death/physiology ; Humans ; Neurons/metabolism
Czasopismo naukowe
Tytuł :
TSPO interacts with VDAC1 and triggers a ROS-mediated inhibition of mitochondrial quality control.
Autorzy :
Gatliff J; a Department of Comparative Biomedical Sciences; The Royal Veterinary College ; University of London ; London , UK.
East D
Crosby J
Abeti R
Harvey R
Craigen W
Parker P
Campanella M
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Źródło :
Autophagy [Autophagy] 2014; Vol. 10 (12), pp. 2279-96.
Typ publikacji :
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms :
Autophagy/*physiology
Mitochondria/*metabolism
Reactive Oxygen Species/*metabolism
Receptors, GABA/*metabolism
Ubiquitination/*physiology
Voltage-Dependent Anion Channel 1/*metabolism
Animals ; Biological Transport/physiology ; Mice ; Mitochondrial Membranes/metabolism ; Ubiquitin-Protein Ligases/metabolism
Czasopismo naukowe
Tytuł :
Inhibition of LRRK2 kinase activity stimulates macroautophagy.
Autorzy :
Manzoni C; Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square, London, WC1N 3BG, UK. Electronic address: .
Mamais A; Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square, London, WC1N 3BG, UK; Reta Lila Weston Institute and Queen Square Brain Bank, UCL Institute of Neurology, 1 Wakefield Street, London, WC1N 1PJ, UK.
Dihanich S; Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square, London, WC1N 3BG, UK.
Abeti R; Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square, London, WC1N 3BG, UK.
Soutar MPM; Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square, London, WC1N 3BG, UK.
Plun-Favreau H; Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square, London, WC1N 3BG, UK.
Giunti P; Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square, London, WC1N 3BG, UK.
Tooze SA; London Research Institute, Cancer Research UK, Lincoln's Inn Fields, London, WC2A 3LY, UK.
Bandopadhyay R; Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square, London, WC1N 3BG, UK; Reta Lila Weston Institute and Queen Square Brain Bank, UCL Institute of Neurology, 1 Wakefield Street, London, WC1N 1PJ, UK.
Lewis PA; Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square, London, WC1N 3BG, UK; School of Pharmacy, University of Reading, Whiteknights, Reading, RG6 6AP, UK. Electronic address: .
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Źródło :
Biochimica et biophysica acta [Biochim Biophys Acta] 2013 Dec; Vol. 1833 (12), pp. 2900-2910. Date of Electronic Publication: 2013 Aug 01.
Typ publikacji :
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms :
Autophagy*/drug effects
Protein-Serine-Threonine Kinases/*antagonists & inhibitors
Adaptor Proteins, Signal Transducing/metabolism ; Animals ; Astrocytes/cytology ; Astrocytes/drug effects ; Astrocytes/enzymology ; Benzodiazepinones/pharmacology ; Cell Line, Tumor ; Gene Knockdown Techniques ; HEK293 Cells ; Humans ; Immunohistochemistry ; Leucine-Rich Repeat Serine-Threonine Protein Kinase-2 ; Lysosomes/drug effects ; Lysosomes/metabolism ; Microtubule-Associated Proteins ; Protein Kinase Inhibitors/pharmacology ; Protein Transport/drug effects ; Protein-Serine-Threonine Kinases/metabolism ; Pyrimidines/pharmacology ; Rats ; Sequestosome-1 Protein
Czasopismo naukowe
Tytuł :
Point mutations in the transmembrane region of the clic1 ion channel selectively modify its biophysical properties.
Autorzy :
Averaimo S; Dipartimento di Bioscienze, Università degli Studi di Milano, Milan, Italy.
Abeti R
Savalli N
Brown LJ
Curmi PM
Breit SN
Mazzanti M
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Źródło :
PloS one [PLoS One] 2013 Sep 18; Vol. 8 (9), pp. e74523. Date of Electronic Publication: 2013 Sep 18 (Print Publication: 2013).
Typ publikacji :
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms :
Biophysical Phenomena*
Cell Membrane/*chemistry
Chloride Channels/*chemistry
Chloride Channels/*genetics
Point Mutation/*genetics
Amino Acid Substitution ; Chloride Channels/metabolism ; Electrophysiological Phenomena ; HEK293 Cells ; Humans ; Lipid Bilayers/metabolism ; Mutant Proteins/chemistry ; Mutant Proteins/metabolism ; Patch-Clamp Techniques ; Structure-Activity Relationship ; Time Factors ; Transfection
Czasopismo naukowe
Tytuł :
Activation of PARP by oxidative stress induced by β-amyloid: implications for Alzheimer's disease.
Autorzy :
Abeti R; Department of Molecular Neuroscience, Institute of Neurology, UCL, Queen Square, London, WC1N 3BG, UK. r.abeti@ucl.ac.uk
Duchen MR
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Źródło :
Neurochemical research [Neurochem Res] 2012 Nov; Vol. 37 (11), pp. 2589-96. Date of Electronic Publication: 2012 Oct 18.
Typ publikacji :
Journal Article; Review
MeSH Terms :
Oxidative Stress*
Alzheimer Disease/*metabolism
Amyloid beta-Peptides/*metabolism
Poly(ADP-ribose) Polymerases/*metabolism
Alzheimer Disease/enzymology ; Animals ; Enzyme Activation ; Humans ; Reactive Oxygen Species/metabolism
Czasopismo naukowe
Tytuł :
IF1, the endogenous regulator of the F(1)F(o)-ATPsynthase, defines mitochondrial volume fraction in HeLa cells by regulating autophagy.
Autorzy :
Campanella M; Department of Cell and Developmental Biology, University College London, UK. />Seraphim A
Abeti R
Casswell E
Echave P
Duchen MR
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Źródło :
Biochimica et biophysica acta [Biochim Biophys Acta] 2009 May; Vol. 1787 (5), pp. 393-401. Date of Electronic Publication: 2009 Mar 05.
Typ publikacji :
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms :
Autophagy/*physiology
HeLa Cells/*metabolism
Proteins/*metabolism
Proton-Translocating ATPases/*metabolism
Reactive Oxygen Species/*metabolism
Adenosine Triphosphate/metabolism ; Cell Death ; Homeostasis ; Humans ; Membrane Potentials/physiology ; Mitochondria/enzymology ; Mitochondria/physiology ; Mitochondria/ultrastructure ; Proteins/genetics ; RNA, Messenger/genetics ; Signal Transduction ; Transcription Factors/genetics ; Transcription Factors/metabolism ; Transfection
Czasopismo naukowe
Tytuł :
Blockade of chloride intracellular ion channel 1 stimulates Abeta phagocytosis.
Autorzy :
Paradisi S; Department of Cell Biology and Neuroscience, Istituto Superiore di Sanità, Rome, Italy.
Matteucci A
Fabrizi C
Denti MA
Abeti R
Breit SN
Malchiodi-Albedi F
Mazzanti M
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Źródło :
Journal of neuroscience research [J Neurosci Res] 2008 Aug 15; Vol. 86 (11), pp. 2488-98.
Typ publikacji :
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms :
Amyloid beta-Peptides/*metabolism
Chloride Channels/*metabolism
Microglia/*metabolism
Phagocytosis/*physiology
Animals ; Cells, Cultured ; Diuretics/pharmacology ; Flow Cytometry ; Glycolates/pharmacology ; Image Processing, Computer-Assisted ; Immunohistochemistry ; Macrophages, Peritoneal/metabolism ; Mice ; Microscopy, Confocal ; Nitric Oxide Synthase Type II/biosynthesis ; Nitric Oxide Synthase Type II/drug effects ; Phagocytosis/drug effects ; RNA Interference ; Rats
Czasopismo naukowe
Tytuł :
Comparison between low-level 50 Hz and 900 MHz electromagnetic stimulation on single channel ionic currents and on firing frequency in dorsal root ganglion isolated neurons.
Autorzy :
Marchionni I; Dipartimento di Biologia Cellulare e dello Sviluppo Università La Sapienza, P.le Aldo Moro 5, I-00185 Roma, Italy.
Paffi A
Pellegrino M
Liberti M
Apollonio F
Abeti R
Fontana F
D'Inzeo G
Mazzanti M
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Źródło :
Biochimica et biophysica acta [Biochim Biophys Acta] 2006 May; Vol. 1758 (5), pp. 597-605. Date of Electronic Publication: 2006 Apr 05.
Typ publikacji :
Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms :
Electric Stimulation*
Electromagnetic Fields*
Ganglia, Spinal/*radiation effects
Ion Channels/*physiology
Neurons/*radiation effects
Action Potentials ; Animals ; Cells, Cultured ; Ganglia, Spinal/cytology ; Ganglia, Spinal/physiology ; Neurons/physiology ; Rats
Czasopismo naukowe
    Wyświetlanie 1-54 z 54

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