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Wyświetlanie 1-50 z 803
Tytuł:
Hsp90 Inhibition: A Promising Therapeutic Approach for ARSACS.
Autorzy:
Nethisinghe S; Ataxia Centre, Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, Queen Square, London WC1N 3BG, UK.
Abeti R; Ataxia Centre, Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, Queen Square, London WC1N 3BG, UK.
Kesavan M; Ataxia Centre, Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, Queen Square, London WC1N 3BG, UK.
Wigley WC; Reata Pharmaceuticals Inc., 2801 Gateway Drive, Irving, TX 75063, USA.
Giunti P; Ataxia Centre, Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, Queen Square, London WC1N 3BG, UK.
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Źródło:
International journal of molecular sciences [Int J Mol Sci] 2021 Oct 29; Vol. 22 (21). Date of Electronic Publication: 2021 Oct 29.
Typ publikacji:
Journal Article
MeSH Terms:
HSP90 Heat-Shock Proteins/*antagonists & inhibitors
Muscle Spasticity/*drug therapy
Novobiocin/*analogs & derivatives
Spinocerebellar Ataxias/*congenital
Cell Line ; Cells, Cultured ; Fibroblasts/drug effects ; Fibroblasts/metabolism ; HSP90 Heat-Shock Proteins/metabolism ; Humans ; Mitochondria/drug effects ; Mitochondria/metabolism ; Muscle Spasticity/metabolism ; Novobiocin/pharmacology ; Spinocerebellar Ataxias/drug therapy ; Spinocerebellar Ataxias/metabolism ; Vimentin/metabolism
SCR Disease Name:
Spastic ataxia Charlevoix-Saguenay type
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
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] 2021 Jan; Vol. 178 (2), pp. 298-311. Date of Electronic Publication: 2020 Dec 14.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Mitochondria*/metabolism
Proton-Translocating ATPases*/metabolism
Adenosine Triphosphate ; Cell Death ; Humans ; Hydrolysis
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
A randomized feasibility trial of medium chain triglyceride-supplemented ketogenic diet in people with Parkinson's disease.
Autorzy:
Choi, Alexander H. (AUTHOR)
Delgado, Melanie (AUTHOR)
Chen, Kong Y. (AUTHOR)
Chung, Stephanie T. (AUTHOR)
Courville, Amber (AUTHOR)
Turner, Sara A. (AUTHOR)
Yang, Shanna (AUTHOR)
Airaghi, Kayla (AUTHOR)
Dustin, Irene (AUTHOR)
McGurrin, Patrick (AUTHOR)
Wu, Tianxia (AUTHOR)
Hallett, Mark (AUTHOR)
Ehrlich, Debra J. (AUTHOR)
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Źródło:
BMC Neurology. 4/1/2024, Vol. 24 Issue 1, p1-19. 19p.
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Poly-(ADP-ribose) polymerases inhibition by olaparib attenuates activities of the NLRP3 inflammasome and of NF-κB in THP-1 monocytes.
Autorzy:
Mustafa, Khamis (AUTHOR)
Han, Ying (AUTHOR)
He, Dan (AUTHOR)
Wang, Ying (AUTHOR)
Niu, Nan (AUTHOR)
Jose, Pedro A. (AUTHOR)
Jiang, Yinong (AUTHOR)
Kopp, Jeffrey B. (AUTHOR)
Lee, Hewang (AUTHOR)
Qu, Peng (AUTHOR)
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Źródło:
PLoS ONE. 2/9/2024, Vol. 19 Issue 2, p1-22. 22p.
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
The Association between Intracranial Calcifications and Symptoms in Patients with Primary Familial Brain Calcification.
Autorzy:
Mathijssen, Gini (AUTHOR)
van Valen, Evelien (AUTHOR)
de Jong, Pim A. (AUTHOR)
Golüke, Nienke M. S. (AUTHOR) r.
van Maren, Emiel A. (AUTHOR)
Snijders, Birgitta M. G. (AUTHOR)
Brilstra, Eva H. (AUTHOR)
Ruigrok, Ynte M. (AUTHOR)
Bakker, Susan (AUTHOR)
Goto, Renzo W. (AUTHOR)
Emmelot-Vonk, Marielle H. (AUTHOR)
Koek, Huiberdina L. (AUTHOR)
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Źródło:
Journal of Clinical Medicine. Feb2024, Vol. 13 Issue 3, p828. 14p.
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
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
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Propensity matched comparison of omaveloxolone treatment to Friedreich ataxia natural history data.
Autorzy:
Lynch, David R. (AUTHOR)
Goldsberry, Angie (AUTHOR)
Rummey, Christian (AUTHOR)
Farmer, Jennifer (AUTHOR)
Boesch, Sylvia (AUTHOR)
Delatycki, Martin B. (AUTHOR)
Giunti, Paola (AUTHOR)
Hoyle, J. Chad (AUTHOR)
Mariotti, Caterina (AUTHOR)
Mathews, Katherine D. (AUTHOR)
Nachbauer, Wolfgang (AUTHOR)
Perlman, Susan (AUTHOR)
Subramony, S.H. (AUTHOR)
Wilmot, George (AUTHOR)
Zesiewicz, Theresa (AUTHOR)
Weissfeld, Lisa (AUTHOR)
Meyer, Colin (AUTHOR)
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Źródło:
Annals of Clinical & Translational Neurology. Jan2024, Vol. 11 Issue 1, p4-16. 13p.
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Fahr's disease linked to a novel mutation in MYORG variants manifesting as paroxysmal limb stiffness and dysarthria: Case report and literature review.
Autorzy:
Zhao, Tianxue (AUTHOR)
Xu, Shaokun (AUTHOR)
Liu, Siyue (AUTHOR)
Xu, Juan (AUTHOR)
Zhang, Xianfeng (AUTHOR)
Zhan, Yuhong (AUTHOR)
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Źródło:
Molecular Genetics & Genomic Medicine. Dec2023, Vol. 11 Issue 12, p1-15. 15p.
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
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
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 ; Tripeptidyl-Peptidase 1 ; Young Adult
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknieSzczegóły
Tytuł:
The Pro-Oncogenic Protein IF 1 Promotes Proliferation of Anoxic Cancer Cells during Re-Oxygenation.
Autorzy:
Righetti, Riccardo (AUTHOR)
Grillini, Silvia (AUTHOR)
Del Dotto, Valentina (AUTHOR)
Costanzini, Anna (AUTHOR)
Liuzzi, Francesca (AUTHOR)
Zanna, Claudia (AUTHOR)
Sgarbi, Gianluca (AUTHOR)
Solaini, Giancarlo (AUTHOR)
Baracca, Alessandra (AUTHOR)
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Źródło:
International Journal of Molecular Sciences. Oct2023, Vol. 24 Issue 19, p14624. 23p.
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Mitochondrial dysfunction in neurodegenerative disorders: Potential therapeutic application of mitochondrial transfer to central nervous system-residing cells.
Autorzy:
Bustamante-Barrientos, Felipe A. (AUTHOR)
Luque-Campos, Noymar (AUTHOR)
Araya, María Jesús (AUTHOR)
Lara-Barba, Eliana (AUTHOR)
de Solminihac, Javiera (AUTHOR)
Pradenas, Carolina (AUTHOR)
Molina, Luis (AUTHOR)
Herrera-Luna, Yeimi (AUTHOR)
Utreras-Mendoza, Yildy (AUTHOR)
Elizondo-Vega, Roberto (AUTHOR)
Vega-Letter, Ana María (AUTHOR)
Luz-Crawford, Patricia (AUTHOR)
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Źródło:
Journal of Translational Medicine. 9/9/2023, Vol. 21 Issue 1, p1-27. 27p.
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
In Vitro Enzymatic Studies Reveal pH and Temperature Sensitive Properties of the CLIC Proteins.
Autorzy:
Alghalayini, Amani (AUTHOR)
Hossain, Khondker Rufaka (AUTHOR)
Moghaddasi, Saba (AUTHOR)
Turkewitz, Daniel R. (AUTHOR) daniel.r.
D'Amario, Claudia (AUTHOR)
Wallach, Michael (AUTHOR)
Valenzuela, Stella M. (AUTHOR)
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Źródło:
Biomolecules (2218-273X). Sep2023, Vol. 13 Issue 9, p1394. 18p.
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Interferon Gamma Enhances Cytoprotective Pathways via Nrf2 and MnSOD Induction in Friedreich's Ataxia Cells.
Autorzy:
Luffarelli, Riccardo (AUTHOR)
Panarello, Luca (AUTHOR)
Quatrana, Andrea (AUTHOR)
Tiano, Francesca (AUTHOR)
Fortuni, Silvia (AUTHOR)
Rufini, Alessandra (AUTHOR)
Malisan, Florence (AUTHOR)
Testi, Roberto (AUTHOR)
Condò, Ivano (AUTHOR)
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Źródło:
International Journal of Molecular Sciences. Aug2023, Vol. 24 Issue 16, p12687. 15p.
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
In Vitro Characterization of Motor Neurons and Purkinje Cells Differentiated from Induced Pluripotent Stem Cells Generated from Patients with Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay.
Autorzy:
Louit, Aurélie (AUTHOR)
Beaudet, Marie-Josée (AUTHOR)
Blais, Mathieu (AUTHOR)
Gros-Louis, François (AUTHOR)
Dupré, Nicolas (AUTHOR)
Berthod, François (AUTHOR)
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Źródło:
Stem Cells International. 4/15/2023, p1-11. 11p.
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Impact of Translocator Protein 18 kDa (TSPO) Deficiency on Mitochondrial Function and the Inflammatory State of Human C20 Microglia Cells.
Autorzy:
Bader, Stefanie (AUTHOR)
Würfel, Thea (AUTHOR)
Jahner, Tatjana (AUTHOR)
Nothdurfter, Caroline (AUTHOR)
Rupprecht, Rainer (AUTHOR)
Milenkovic, Vladimir M. (AUTHOR)
Wetzel, Christian H. (AUTHOR)
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Źródło:
Cells (2073-4409). Mar2023, Vol. 12 Issue 6, p954. 16p.
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Exploring mitochondrial biomarkers for Friedreich's ataxia: a multifaceted approach
Autorzy:
Stovickova, LucieAff1, Aff7, IDs00415024122235_cor1
Hansikova, Hana
Hanzalova, Jitka
Musova, ZuzanaAff4, Aff7
Semjonov, Valerij
Stovicek, Pavel
Hadzic, Haris
Novotna, Ludmila
Simcik, Martin
Strnad, Pavel
Serbina, Anastaziia
Karamazovova, SimonaAff6, Aff7
Schwabova Paulasova, JaroslavaAff6, Aff7
Vyhnalek, MartinAff6, Aff7
Krsek, Pavel
Zumrova, AlenaAff1, Aff7
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Źródło:
Journal of Neurology. :1-16
Czasopismo naukowe
Szczegóły
Tytuł:
Identification of a mechanism promoting mitochondrial sterol accumulation during myocardial ischemia–reperfusion: role of TSPO and STAR
Autorzy:
Bréhat, Juliette
Leick, Shirin
Musman, Julien
Su, Jin Bo
Eychenne, Nicolas
Giton, Frank
Rivard, Michael
Barel, Louis-Antoine
Tropeano, Chiara
Vitarelli, Frederica
Caccia, Claudio
Leoni, Valerio
Ghaleh, Bijan
Pons, Sandrine
Morin, DidierAff1, IDs00395024010433_cor15
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Źródło:
Basic Research in Cardiology. :1-23
Czasopismo naukowe
Szczegóły
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
Pełny tekst  Link otwiera się w nowym oknieSzczegóły
Tytuł:
Autosomal recessive cerebellar ataxias: a diagnostic classification approach according to ocular features.
Autorzy:
Lopergolo, Diego
Rosini, Francesca
Pretegiani, Elena
Bargagli, Alessia
Serchi, Valeria
Rufa, Alessandra
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Temat:
EYE tracking
EYE movements
COMPUTER science
MACHINE learning
OPTIC nerve
NEURODEGENERATION
FAMILIAL spastic paraplegia
CEREBELLAR cortex
Źródło:
Frontiers in Integrative Neuroscience; 2024, p1-17, 17p
Czasopismo naukowe
Szczegóły
Wyświetlanie 1-50 z 803

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