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Wyszukujesz frazę ""Huntingtin Protein"" wg kryterium: Temat


Tytuł:
Huntingtin HTT1a is generated in a CAG repeat-length-dependent manner in human tissues.
Autorzy:
Hoschek F; Department of Neurology, University Hospital Ulm, 89081, Ulm, Germany.
Natan J; Department of Neurology, University Hospital Ulm, 89081, Ulm, Germany.
Wagner M; Department of Neurology, University Hospital Ulm, 89081, Ulm, Germany.
Sathasivam K; Huntington's Disease Centre, Department of Neurodegenerative Disease, Queen Square Institute of Neurology, University College London, WC1N 3BG, London, UK.
Abdelmoez A; Department of Neurology, University Hospital Ulm, 89081, Ulm, Germany.; Department of Pharmaceutical Organic Chemistry, Faculty of Pharmacy, Assiut University, Assiut, Egypt.
von Einem B; Department of Neurology, University Hospital Ulm, 89081, Ulm, Germany.
Bates GP; Huntington's Disease Centre, Department of Neurodegenerative Disease, Queen Square Institute of Neurology, University College London, WC1N 3BG, London, UK.
Landwehrmeyer GB; Department of Neurology, University Hospital Ulm, 89081, Ulm, Germany.
Neueder A; Department of Neurology, University Hospital Ulm, 89081, Ulm, Germany. .
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Źródło:
Molecular medicine (Cambridge, Mass.) [Mol Med] 2024 Mar 08; Vol. 30 (1), pp. 36. Date of Electronic Publication: 2024 Mar 08.
Typ publikacji:
Journal Article
MeSH Terms:
Huntingtin Protein*/genetics
Huntingtin Protein*/metabolism
Huntington Disease*/genetics
Huntington Disease*/metabolism
Trinucleotide Repeat Expansion*
Adult ; Animals ; Humans ; Mice ; Exons/genetics ; Neurons/metabolism ; RNA/metabolism
Czasopismo naukowe
Tytuł:
Fluorescent protein tagging promotes phase separation and alters the aggregation pathway of huntingtin exon-1.
Autorzy:
Pandey NK; Physiology and Neuroscience, Zilkha Neurogenetic Institute, Keck School of Medicine, University of Southern California, Los Angeles, California, USA.
Varkey J; Physiology and Neuroscience, Zilkha Neurogenetic Institute, Keck School of Medicine, University of Southern California, Los Angeles, California, USA.
Ajayan A; Physiology and Neuroscience, Zilkha Neurogenetic Institute, Keck School of Medicine, University of Southern California, Los Angeles, California, USA.
George G; Physiology and Neuroscience, Zilkha Neurogenetic Institute, Keck School of Medicine, University of Southern California, Los Angeles, California, USA.
Chen J; Physiology and Neuroscience, Zilkha Neurogenetic Institute, Keck School of Medicine, University of Southern California, Los Angeles, California, USA.
Langen R; Physiology and Neuroscience, Zilkha Neurogenetic Institute, Keck School of Medicine, University of Southern California, Los Angeles, California, USA; Biochemistry and Molecular Medicine, Zilkha Neurogenetic Institute, Keck School of Medicine, University of Southern California, Los Angeles, California, USA. Electronic address: .
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Źródło:
The Journal of biological chemistry [J Biol Chem] 2024 Jan; Vol. 300 (1), pp. 105585. Date of Electronic Publication: 2023 Dec 21.
Typ publikacji:
Journal Article
MeSH Terms:
Artifacts*
Exons*/genetics
Huntingtin Protein*/chemistry
Huntingtin Protein*/genetics
Huntingtin Protein*/metabolism
Huntington Disease*/genetics
Huntington Disease*/metabolism
Luminescent Measurements*/methods
Phase Separation*
Protein Aggregates*
Red Fluorescent Protein*/genetics
Red Fluorescent Protein*/metabolism
Humans ; Electron Spin Resonance Spectroscopy ; Fluorescence ; Fluorescence Recovery After Photobleaching ; Reproducibility of Results
Czasopismo naukowe
Tytuł:
Interaction between huntingtin exon 1 and HEAT repeat structure probed by chimeric model proteins.
Autorzy:
Zhang H; National Laboratory of Biomacromolecules, CAS Center for Excellence in Biomacromolecules, Institute of Biophysics, Chinese Academy of Sciences, Beijing, China.; University of the Chinese Academy of Sciences, Beijing, China.; Institute of Basic Medical Sciences, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
Wu S; National Laboratory of Biomacromolecules, CAS Center for Excellence in Biomacromolecules, Institute of Biophysics, Chinese Academy of Sciences, Beijing, China.; University of the Chinese Academy of Sciences, Beijing, China.
Itzhaki LS; Department of Pharmacology, University of Cambridge, Cambridge, UK.
Perrett S; National Laboratory of Biomacromolecules, CAS Center for Excellence in Biomacromolecules, Institute of Biophysics, Chinese Academy of Sciences, Beijing, China.; University of the Chinese Academy of Sciences, Beijing, China.
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Źródło:
Protein science : a publication of the Protein Society [Protein Sci] 2023 Dec; Vol. 32 (12), pp. e4810.
Typ publikacji:
Journal Article
MeSH Terms:
Huntingtin Protein*/genetics
Huntingtin Protein*/chemistry
Huntingtin Protein*/metabolism
Exons
Czasopismo naukowe
Tytuł:
Delineation of functional subdomains of Huntingtin protein and their interaction with HAP40.
Autorzy:
Alteen MG; Structural Genomics Consortium, University of Toronto, Toronto, ON M5G 1L7, Canada; POINT Biopharma, 22 St Clair Avenue E Suite 1201, Toronto, ON M4T 2S3, Canada.
Deme JC; Center for Structural Biology, Center for Cancer Research, National Cancer Institute, Frederick, MD 21702, USA.
Alvarez CP; Structural Genomics Consortium, University of Toronto, Toronto, ON M5G 1L7, Canada; SCIEX, 71 Four Valley Dr, Vaughan, ON L4K 4V8, Canada.
Loppnau P; Structural Genomics Consortium, University of Toronto, Toronto, ON M5G 1L7, Canada.
Hutchinson A; Structural Genomics Consortium, University of Toronto, Toronto, ON M5G 1L7, Canada.
Seitova A; Structural Genomics Consortium, University of Toronto, Toronto, ON M5G 1L7, Canada.
Chandrasekaran R; Structural Genomics Consortium, University of Toronto, Toronto, ON M5G 1L7, Canada.
Silva Ramos E; Neuroproteomics, Max Delbrück Center for Molecular Medicine, Robert-Rössle-Str. 10, 13125 Berlin, Germany.
Secker C; Neuroproteomics, Max Delbrück Center for Molecular Medicine, Robert-Rössle-Str. 10, 13125 Berlin, Germany.
Alqazzaz M; Princess Margaret Cancer Centre and Department of Medical Biophysics, University of Toronto, Toronto, ON M5G 1L7, Canada.
Wanker EE; Neuroproteomics, Max Delbrück Center for Molecular Medicine, Robert-Rössle-Str. 10, 13125 Berlin, Germany.
Lea SM; Center for Structural Biology, Center for Cancer Research, National Cancer Institute, Frederick, MD 21702, USA.
Arrowsmith CH; Structural Genomics Consortium, University of Toronto, Toronto, ON M5G 1L7, Canada; Princess Margaret Cancer Centre and Department of Medical Biophysics, University of Toronto, Toronto, ON M5G 1L7, Canada. Electronic address: .
Harding RJ; Structural Genomics Consortium, University of Toronto, Toronto, ON M5G 1L7, Canada; Department of Pharmacology and Toxicology, University of Toronto, Toronto, ON M5S 1A8, Canada. Electronic address: .
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Źródło:
Structure (London, England : 1993) [Structure] 2023 Sep 07; Vol. 31 (9), pp. 1121-1131.e6. Date of Electronic Publication: 2023 Jun 29.
Typ publikacji:
Journal Article; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't
MeSH Terms:
Huntingtin Protein*/chemistry
Nuclear Proteins*/chemistry
Cryoelectron Microscopy ; Humans
Czasopismo naukowe
Tytuł:
Molecular basis of Q-length selectivity for the MW1 antibody-huntingtin interaction.
Autorzy:
Bravo-Arredondo JM; Physiology and Neuroscience, Zilkha Neurogenetic Institute, Keck School of Medicine, University of Southern California, Los Angeles, California, USA.
Venkataraman R; Physiology and Neuroscience, Zilkha Neurogenetic Institute, Keck School of Medicine, University of Southern California, Los Angeles, California, USA.
Varkey J; Physiology and Neuroscience, Zilkha Neurogenetic Institute, Keck School of Medicine, University of Southern California, Los Angeles, California, USA.
Isas JM; Physiology and Neuroscience, Zilkha Neurogenetic Institute, Keck School of Medicine, University of Southern California, Los Angeles, California, USA.
Situ AJ; Physiology and Neuroscience, Zilkha Neurogenetic Institute, Keck School of Medicine, University of Southern California, Los Angeles, California, USA.
Xu H; Physiology and Neuroscience, Zilkha Neurogenetic Institute, Keck School of Medicine, University of Southern California, Los Angeles, California, USA.
Chen J; Physiology and Neuroscience, Zilkha Neurogenetic Institute, Keck School of Medicine, University of Southern California, Los Angeles, California, USA.
Ulmer TS; Physiology and Neuroscience, Zilkha Neurogenetic Institute, Keck School of Medicine, University of Southern California, Los Angeles, California, USA; Biochemistry and Molecular Medicine, Zilkha Neurogenetic Institute, Keck School of Medicine, University of Southern California, Los Angeles, California, USA.
Langen R; Physiology and Neuroscience, Zilkha Neurogenetic Institute, Keck School of Medicine, University of Southern California, Los Angeles, California, USA; Biochemistry and Molecular Medicine, Zilkha Neurogenetic Institute, Keck School of Medicine, University of Southern California, Los Angeles, California, USA. Electronic address: .
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Źródło:
The Journal of biological chemistry [J Biol Chem] 2023 Apr; Vol. 299 (4), pp. 104616. Date of Electronic Publication: 2023 Mar 16.
Typ publikacji:
Journal Article; Research Support, N.I.H., Extramural
MeSH Terms:
Antibodies, Monoclonal*/metabolism
Huntingtin Protein*/chemistry
Huntingtin Protein*/genetics
Huntingtin Protein*/metabolism
Humans ; Exons/genetics ; Huntington Disease/genetics ; Protein Conformation, alpha-Helical/genetics ; Protein Binding ; Magnetic Resonance Spectroscopy ; Protein Multimerization/genetics
Czasopismo naukowe
Tytuł:
Retinal dysfunction in Huntington's disease mouse models concurs with local gliosis and microglia activation.
Autorzy:
Cano-Cano F; Instituto de Investigación e Innovación Biomédica de Cádiz (INiBICA), Unidad de Investigación, Hospital Universitario Puerta del Mar, Av. Ana de Viya 21, 11009, Cádiz, Spain.
Martín-Loro F; Instituto de Investigación e Innovación Biomédica de Cádiz (INiBICA), Unidad de Investigación, Hospital Universitario Puerta del Mar, Av. Ana de Viya 21, 11009, Cádiz, Spain.
Gallardo-Orihuela A; Instituto de Investigación e Innovación Biomédica de Cádiz (INiBICA), Unidad de Investigación, Hospital Universitario Puerta del Mar, Av. Ana de Viya 21, 11009, Cádiz, Spain.
González-Montelongo MDC; Instituto de Investigación e Innovación Biomédica de Cádiz (INiBICA), Unidad de Investigación, Hospital Universitario Puerta del Mar, Av. Ana de Viya 21, 11009, Cádiz, Spain.
Ortuño-Miquel S; Instituto de Investigación Sanitaria y Biomédica de Alicante (ISABIAL), Unidad de Bioinformática, Hospital General Universitario Dr. Balmis, 03010, Alicante, Spain.
Hervás-Corpión I; Instituto de Investigación e Innovación Biomédica de Cádiz (INiBICA), Unidad de Investigación, Hospital Universitario Puerta del Mar, Av. Ana de Viya 21, 11009, Cádiz, Spain.; Programa de Tumores Sólidos, Centro de Investigación Médica Aplicada (CIMA), Departamento de Pediatría, Clínica Universidad de Navarra, Instituto de Investigación Sanitaria de Navarra (IdiSNA), 31008, Pamplona, Spain.
de la Villa P; Departamento de Biología de Sistemas, Universidad de Alcalá de Henares, 28871, Alcalá de Henares, Spain.; Instituto Ramón y Cajal de Investigación Sanitaria (IRYCIS), 28034, Madrid, Spain.
Ramón-Marco L; Laboratorio de Investigación, Diagnostics Building, Instituto de Investigación Sanitaria y Biomédica de Alicante (ISABIAL), Hospital General Universitario Dr. Balmis, Av. Pintor Baeza 12, 03010, Alicante, Spain.
Navarro-Calvo J; Laboratorio de Investigación, Diagnostics Building, Instituto de Investigación Sanitaria y Biomédica de Alicante (ISABIAL), Hospital General Universitario Dr. Balmis, Av. Pintor Baeza 12, 03010, Alicante, Spain.
Gómez-Jaramillo L; Instituto de Investigación e Innovación Biomédica de Cádiz (INiBICA), Unidad de Investigación, Hospital Universitario Puerta del Mar, Av. Ana de Viya 21, 11009, Cádiz, Spain.
Arroba AI; Instituto de Investigación e Innovación Biomédica de Cádiz (INiBICA), Unidad de Investigación, Hospital Universitario Puerta del Mar, Av. Ana de Viya 21, 11009, Cádiz, Spain. .
Valor LM; Laboratorio de Investigación, Diagnostics Building, Instituto de Investigación Sanitaria y Biomédica de Alicante (ISABIAL), Hospital General Universitario Dr. Balmis, Av. Pintor Baeza 12, 03010, Alicante, Spain. valor_.; Instituto de Investigación, Desarrollo e Innovación en Biotecnología Sanitaria de Elche (IDiBE), 03202, Elche, Spain. valor_.
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Źródło:
Scientific reports [Sci Rep] 2024 Feb 20; Vol. 14 (1), pp. 4176. Date of Electronic Publication: 2024 Feb 20.
Typ publikacji:
Journal Article
MeSH Terms:
Huntington Disease*/pathology
Mice ; Animals ; Humans ; Mice, Transgenic ; Gliosis/genetics ; Gliosis/pathology ; Microglia/metabolism ; Neuroinflammatory Diseases ; Disease Models, Animal ; Corpus Striatum/metabolism ; Huntingtin Protein/genetics ; Huntingtin Protein/metabolism
Czasopismo naukowe
Tytuł:
CHCHD2 up-regulation in Huntington disease mediates a compensatory protective response against oxidative stress.
Autorzy:
Liu X; Department of Neurology and Stroke Center, The First Affiliated Hospital of Jinan University, 613 Huangpu Avenue West, Guangzhou, Guangdong, 510632, China.; Clinical Neuroscience Institute, Jinan University, 613 Huangpu Avenue West, Guangzhou, Guangdong, 510632, China.; Department of Neurology, Taihe Hospital of Shiyan, Affiliated Hospital of Hubei Medical University, Shiyan, 442000, China.
Wang F; Department of Neurology and Stroke Center, The First Affiliated Hospital of Jinan University, 613 Huangpu Avenue West, Guangzhou, Guangdong, 510632, China.; Clinical Neuroscience Institute, Jinan University, 613 Huangpu Avenue West, Guangzhou, Guangdong, 510632, China.
Fan X; Department of Neurology and Stroke Center, The First Affiliated Hospital of Jinan University, 613 Huangpu Avenue West, Guangzhou, Guangdong, 510632, China.; Clinical Neuroscience Institute, Jinan University, 613 Huangpu Avenue West, Guangzhou, Guangdong, 510632, China.
Chen M; Department of Neurology and Stroke Center, The First Affiliated Hospital of Jinan University, 613 Huangpu Avenue West, Guangzhou, Guangdong, 510632, China.; Clinical Neuroscience Institute, Jinan University, 613 Huangpu Avenue West, Guangzhou, Guangdong, 510632, China.
Xu X; Department of Neurology and Stroke Center, The First Affiliated Hospital of Jinan University, 613 Huangpu Avenue West, Guangzhou, Guangdong, 510632, China.; Clinical Neuroscience Institute, Jinan University, 613 Huangpu Avenue West, Guangzhou, Guangdong, 510632, China.
Xu Q; Department of Plastic Surgery, The First Affiliated Hospital, Jinan University, 613 Huangpu Avenue West, Guangzhou, Guangdong, 510632, China.
Zhu H; Department of Neurology and Stroke Center, The First Affiliated Hospital of Jinan University, 613 Huangpu Avenue West, Guangzhou, Guangdong, 510632, China.
Xu A; Department of Neurology and Stroke Center, The First Affiliated Hospital of Jinan University, 613 Huangpu Avenue West, Guangzhou, Guangdong, 510632, China.; Clinical Neuroscience Institute, Jinan University, 613 Huangpu Avenue West, Guangzhou, Guangdong, 510632, China.
Pouladi MA; Department of Medical Genetics, Centre for Molecular Medicine and Therapeutics, Djavad Mowafaghian Centre for Brain Health, British Columbia Children's Hospital Research Institute, University of British Columbia, Vancouver, V5Z 4H4, Canada. .
Xu X; Department of Neurology and Stroke Center, The First Affiliated Hospital of Jinan University, 613 Huangpu Avenue West, Guangzhou, Guangdong, 510632, China. .; Clinical Neuroscience Institute, Jinan University, 613 Huangpu Avenue West, Guangzhou, Guangdong, 510632, China. .
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Źródło:
Cell death & disease [Cell Death Dis] 2024 Feb 10; Vol. 15 (2), pp. 126. Date of Electronic Publication: 2024 Feb 10.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Huntington Disease*/metabolism
Neurodegenerative Diseases*/metabolism
Induced Pluripotent Stem Cells*/metabolism
Animals ; Mice ; Humans ; Up-Regulation/genetics ; Oxidative Stress ; Huntingtin Protein/genetics ; Huntingtin Protein/metabolism ; DNA-Binding Proteins/genetics ; DNA-Binding Proteins/metabolism ; Transcription Factors/genetics ; Transcription Factors/metabolism
Czasopismo naukowe
Tytuł:
Unraveling the Puzzle of Therapeutic Peptides: A Promising Frontier in Huntington's Disease Treatment.
Autorzy:
Ahamad S; Department of Chemistry, Aligarh Muslim University, Aligarh 202002, India.
Bano N; Department of Zoology, Aligarh Muslim University, Aligarh 202002, India.
Khan S; Department of Zoology, Aligarh Muslim University, Aligarh 202002, India.
Hussain MK; Department of Chemistry, Govt. Raza P.G. College, Rampur 244901, India.
Bhat SA; Department of Zoology, Aligarh Muslim University, Aligarh 202002, India.
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Źródło:
Journal of medicinal chemistry [J Med Chem] 2024 Jan 25; Vol. 67 (2), pp. 783-815. Date of Electronic Publication: 2024 Jan 11.
Typ publikacji:
Journal Article; Review
MeSH Terms:
Huntington Disease*/drug therapy
Huntington Disease*/genetics
Humans ; Animals ; Signal Transduction ; Peptides/pharmacology ; Peptides/therapeutic use ; Huntingtin Protein/genetics ; Huntingtin Protein/metabolism ; Disease Models, Animal
Czasopismo naukowe
Tytuł:
Altered anterograde axonal transport of mitochondria in cultured striatal neurons of a knock-in mouse model of Huntington's disease.
Autorzy:
Wu C; Division of Life Science, The Hong Kong University of Science and Technology, Clear Water Bay, Kowloon, Hong Kong.
Yin H; Division of Life Science, The Hong Kong University of Science and Technology, Clear Water Bay, Kowloon, Hong Kong.
Fu S; Division of Life Science, The Hong Kong University of Science and Technology, Clear Water Bay, Kowloon, Hong Kong.
Yoo H; Division of Life Science, The Hong Kong University of Science and Technology, Clear Water Bay, Kowloon, Hong Kong.
Zhang M; Division of Life Science, The Hong Kong University of Science and Technology, Clear Water Bay, Kowloon, Hong Kong.
Park H; Division of Life Science, The Hong Kong University of Science and Technology, Clear Water Bay, Kowloon, Hong Kong; Department of Physics, The Hong Kong University of Science and Technology, Clear Water Bay, Kowloon, Hong Kong; State Key Laboratory of Molecular Neuroscience, The Hong Kong University of Science and Technology, Clear Water Bay, Kowloon, Hong Kong. Electronic address: .
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Źródło:
Biochemical and biophysical research communications [Biochem Biophys Res Commun] 2024 Jan 08; Vol. 691, pp. 149246. Date of Electronic Publication: 2023 Nov 18.
Typ publikacji:
Journal Article
MeSH Terms:
Huntington Disease*/metabolism
Neurodegenerative Diseases*/metabolism
Mice ; Animals ; Axonal Transport ; Mice, Transgenic ; Neurons/metabolism ; Corpus Striatum/metabolism ; Disease Models, Animal ; Mitochondria/metabolism ; Huntingtin Protein/genetics ; Huntingtin Protein/metabolism
Czasopismo naukowe
Tytuł:
Antisense oligonucleotide-mediated disruption of HTT caspase-6 cleavage site ameliorates the phenotype of YAC128 Huntington disease mice.
Autorzy:
Kuijper EC; Department of Human Genetics, Leiden University Medical Center, the Netherlands. Electronic address: .
Overzier M; Department of Human Genetics, Leiden University Medical Center, the Netherlands.
Suidgeest E; Department of Radiology, Leiden University Medical Center, the Netherlands.
Dzyubachyk O; Department of Radiology, Leiden University Medical Center, the Netherlands.
Maguin C; Université Paris-Saclay, Commissariat à l'Energie Atomique et aux Energies Alternatives, Centre National de la Recherche Scientifique, Molecular Imaging Research Center, Laboratoire des Maladies Neurodégénératives, France.
Pérot JB; Université Paris-Saclay, Commissariat à l'Energie Atomique et aux Energies Alternatives, Centre National de la Recherche Scientifique, Molecular Imaging Research Center, Laboratoire des Maladies Neurodégénératives, France; Institut du Cerveau - Paris Brain Institute, Sorbonne Université, France.
Flament J; Université Paris-Saclay, Commissariat à l'Energie Atomique et aux Energies Alternatives, Centre National de la Recherche Scientifique, Molecular Imaging Research Center, Laboratoire des Maladies Neurodégénératives, France.
Ariyurek Y; Department of Human Genetics, Leiden University Medical Center, the Netherlands.
Mei H; Department of Biomedical Data Sciences, Leiden University Medical Center, the Netherlands.
Buijsen RAM; Department of Human Genetics, Leiden University Medical Center, the Netherlands.
van der Weerd L; Department of Human Genetics, Leiden University Medical Center, the Netherlands; Department of Radiology, Leiden University Medical Center, the Netherlands.
van Roon-Mom W; Department of Human Genetics, Leiden University Medical Center, the Netherlands.
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Źródło:
Neurobiology of disease [Neurobiol Dis] 2024 Jan; Vol. 190, pp. 106368. Date of Electronic Publication: 2023 Nov 29.
Typ publikacji:
Journal Article
MeSH Terms:
Huntington Disease*/metabolism
Animals ; Humans ; Mice ; Caspase 6/genetics ; Caspase 6/metabolism ; Corpus Striatum/metabolism ; Huntingtin Protein/genetics ; Huntingtin Protein/metabolism ; Oligonucleotides, Antisense/pharmacology ; Phenotype
Czasopismo naukowe
Tytuł:
Dose-dependent reduction of somatic expansions but not Htt aggregates by di-valent siRNA-mediated silencing of MSH3 in HdhQ111 mice.
Autorzy:
Driscoll, Rachelle (AUTHOR)
Hampton, Lucas (AUTHOR)
Abraham, Neeta A. (AUTHOR)
Larigan, J. Douglas (AUTHOR)
Joseph, Nadine F. (AUTHOR)
Hernandez-Vega, Juan C. (AUTHOR)
Geisler, Sarah (AUTHOR)
Yang, Fu-Chia (AUTHOR)
Deninger, Matthew (AUTHOR)
Tran, David T. (AUTHOR)
Khatri, Natasha (AUTHOR)
Godinho, Bruno M. D. C. (AUTHOR)
Kinberger, Garth A. (AUTHOR)
Montagna, Daniel R. (AUTHOR)
Hirst, Warren D. (AUTHOR)
Guardado, Catherine L. (AUTHOR)
Glajch, Kelly E. (AUTHOR)
Arnold, H. Moore (AUTHOR)
Gallant-Behm, Corrie L. (AUTHOR)
Weihofen, Andreas (AUTHOR)
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Źródło:
Scientific Reports. 1/24/2024, Vol. 14 Issue 1, p1-11. 11p.
Czasopismo naukowe
Tytuł:
Reducing huntingtin by immunotherapy delays disease progression in a mouse model of Huntington disease.
Autorzy:
Bartl S; HD Immune, Vienna, Austria. Electronic address: .
Xie Y; University of Central Florida, Burnett School of Biomedical Sciences, Orlando, FL, United States of America; University of British Columbia, Centre for Molecular Medicine and Therapeutics, Vancouver, Canada.
Potluri N; University of Central Florida, Burnett School of Biomedical Sciences, Orlando, FL, United States of America.
Kesineni R; University of Central Florida, Burnett School of Biomedical Sciences, Orlando, FL, United States of America.
Hencak K; University of Central Florida, Burnett School of Biomedical Sciences, Orlando, FL, United States of America.
Cengio LD; University of British Columbia, Centre for Molecular Medicine and Therapeutics, Vancouver, Canada.
Balazs K; AFFiRiS AG, Vienna, Austria.
Oueslati A; Centre de recherche du CHU - Université Laval, Québec, Canada.
Parth M; AFFiRiS AG, Vienna, Austria.
Salhat N; AFFiRiS AG, Vienna, Austria.
Siddu A; Centre de recherche du CHU - Université Laval, Québec, Canada.
Smrzka O; AFFiRiS AG, Vienna, Austria.
Cicchetti F; Centre de recherche du CHU - Université Laval, Québec, Canada.
Straffler G; AFFiRiS AG, Vienna, Austria.
Hayden MR; University of British Columbia, Centre for Molecular Medicine and Therapeutics, Vancouver, Canada.
Southwell AL; University of Central Florida, Burnett School of Biomedical Sciences, Orlando, FL, United States of America. Electronic address: .
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Źródło:
Neurobiology of disease [Neurobiol Dis] 2024 Jan; Vol. 190, pp. 106376. Date of Electronic Publication: 2023 Dec 12.
Typ publikacji:
Journal Article
MeSH Terms:
Huntington Disease*/metabolism
Mice ; Animals ; Mice, Transgenic ; Nerve Tissue Proteins/metabolism ; Nuclear Proteins/metabolism ; Huntingtin Protein/genetics ; Immunotherapy ; Disease Models, Animal ; Antibodies, Monoclonal/pharmacology ; Antibodies, Monoclonal/therapeutic use ; Disease Progression
Czasopismo naukowe
Tytuł:
Analysis of HTT CAG repeat expansion among healthy individuals and patients with chorea in Korea.
Autorzy:
Kim R; Department of Neurology, Inha University Hospital, Inha University College of Medicine, Incheon, South Korea.
Seong MW; Department of Laboratory Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, South Korea; Cancer Research Institute, Seoul National University College of Medicine, Seoul, South Korea.
Oh B; Department of Familial Medicine, Seoul Metropolitan Government-Seoul National University Boramae Medical Center, Seoul National University College of Medicine, Seoul, South Korea.
Shin HS; Department of Laboratory Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, South Korea.
Lee JS; Department of Laboratory Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, South Korea.
Park S; Department of Neurology, Daejeon Eulji Medical Center, Eulji University College of Medicine, Daejeon, South Korea.
Jang M; Department of Neurology, JMH Seoul Neurologic Clinic, Seoul, South Korea.
Jeon B; Department of Neurology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, South Korea.
Kim HJ; Department of Neurology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, South Korea. Electronic address: .
Lee JY; Department of Neurology, Seoul Metropolitan Government - Seoul National University Boramae Medical Center, Seoul National University College of Medicine, Seoul, South Korea. Electronic address: .
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Źródło:
Parkinsonism & related disorders [Parkinsonism Relat Disord] 2024 Jan; Vol. 118, pp. 105930. Date of Electronic Publication: 2023 Nov 21.
Typ publikacji:
Journal Article
MeSH Terms:
Chorea*/genetics
Huntington Disease*/genetics
Humans ; Aged ; Alleles ; Gene Frequency ; Huntingtin Protein/genetics ; Republic of Korea/epidemiology ; Trinucleotide Repeat Expansion/genetics
Czasopismo naukowe
Tytuł:
Glucocorticoid receptor antagonist CORT113176 attenuates motor and neuropathological symptoms of Huntington's disease in R6/2 mice.
Autorzy:
Gentenaar M; Department of Medicine, Division of Endocrinology, Leiden University Medical Center, Leiden, the Netherlands. Electronic address: .
Meulmeester FL; Department of Medicine, Division of Endocrinology, Leiden University Medical Center, Leiden, the Netherlands.
van der Burg XR; Department of Medicine, Division of Endocrinology, Leiden University Medical Center, Leiden, the Netherlands.
Hoekstra AT; Department of Medicine, Division of Endocrinology, Leiden University Medical Center, Leiden, the Netherlands; Center for Proteomics and Metabolomics, Leiden University Medical Center, Leiden, the Netherlands.
Hunt H; Corcept Therapeutics, Menlo Park, CA, USA.
Kroon J; Department of Medicine, Division of Endocrinology, Leiden University Medical Center, Leiden, the Netherlands; Corcept Therapeutics, Menlo Park, CA, USA.
van Roon-Mom WMC; Department of Human Genetics, Leiden University Medical Center, Leiden, the Netherlands.
Meijer OC; Department of Medicine, Division of Endocrinology, Leiden University Medical Center, Leiden, the Netherlands.
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Źródło:
Experimental neurology [Exp Neurol] 2024 Apr; Vol. 374, pp. 114675. Date of Electronic Publication: 2024 Jan 10.
Typ publikacji:
Journal Article
MeSH Terms:
Huntington Disease*/complications
Huntington Disease*/drug therapy
Huntington Disease*/genetics
Isoquinolines*
Neurodegenerative Diseases*
Pyrazoles*
Animals ; Female ; Humans ; Male ; Mice ; Disease Models, Animal ; Huntingtin Protein/genetics ; Huntingtin Protein/metabolism ; Receptors, Glucocorticoid
Czasopismo naukowe
Tytuł:
Differential expression and roles of Huntingtin and Huntingtin-associated protein 1 in the mouse and primate brains.
Autorzy:
Chen X; Brain Science and Advanced Technology Institute, Wuhan University of Science and Technology, Wuhan, 430000, Hubei, China.; Guangdong Key Laboratory of Non-Human Primate Research, Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, Guangzhou, 510632, China.
Sun Y; Guangdong Key Laboratory of Non-Human Primate Research, Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, Guangzhou, 510632, China.
Chen L; Guangdong Key Laboratory of Non-Human Primate Research, Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, Guangzhou, 510632, China.
Chen XS; Guangdong Key Laboratory of Non-Human Primate Research, Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, Guangzhou, 510632, China.
Pan M; Guangdong Key Laboratory of Non-Human Primate Research, Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, Guangzhou, 510632, China.
Zhang Y; Guangdong Key Laboratory of Non-Human Primate Research, Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, Guangzhou, 510632, China.
Wang Q; Guangdong Key Laboratory of Non-Human Primate Research, Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, Guangzhou, 510632, China.
Yang W; Guangdong Key Laboratory of Non-Human Primate Research, Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, Guangzhou, 510632, China.
Yin P; Guangdong Key Laboratory of Non-Human Primate Research, Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, Guangzhou, 510632, China.
He D; Guangdong Key Laboratory of Non-Human Primate Research, Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, Guangzhou, 510632, China.
Guo X; Guangdong Key Laboratory of Non-Human Primate Research, Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, Guangzhou, 510632, China.
Yang S; Guangdong Key Laboratory of Non-Human Primate Research, Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, Guangzhou, 510632, China.
Zeng Y; Brain Science and Advanced Technology Institute, Wuhan University of Science and Technology, Wuhan, 430000, Hubei, China.
Yan S; Guangdong Key Laboratory of Non-Human Primate Research, Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, Guangzhou, 510632, China.
Li XJ; Guangdong Key Laboratory of Non-Human Primate Research, Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, Guangzhou, 510632, China. .
Li S; Guangdong Key Laboratory of Non-Human Primate Research, Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, Guangzhou, 510632, China. .
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Źródło:
Cellular and molecular life sciences : CMLS [Cell Mol Life Sci] 2022 Oct 17; Vol. 79 (11), pp. 554. Date of Electronic Publication: 2022 Oct 17.
Typ publikacji:
Journal Article
MeSH Terms:
Huntingtin Protein*/genetics
Huntingtin Protein*/metabolism
Huntington Disease*/genetics
Huntington Disease*/metabolism
Nerve Tissue Proteins*/genetics
Nerve Tissue Proteins*/metabolism
Animals ; Humans ; Mice ; Brain/metabolism ; Neurons/metabolism ; Primates/genetics ; Primates/metabolism
Czasopismo naukowe
Tytuł:
Elucidation of Site-Specific Ubiquitination on Chaperones in Response to Mutant Huntingtin.
Autorzy:
Panda P; School of Biosciences and Bioengineering, Indian Institute of Technology Mandi, Kamand, Himachal Pradesh, 175005, India.
Sarohi V; School of Biosciences and Bioengineering, Indian Institute of Technology Mandi, Kamand, Himachal Pradesh, 175005, India.
Basak T; School of Biosciences and Bioengineering, Indian Institute of Technology Mandi, Kamand, Himachal Pradesh, 175005, India. .
Kasturi P; School of Biosciences and Bioengineering, Indian Institute of Technology Mandi, Kamand, Himachal Pradesh, 175005, India. .
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Źródło:
Cellular and molecular neurobiology [Cell Mol Neurobiol] 2023 Dec 15; Vol. 44 (1), pp. 3. Date of Electronic Publication: 2023 Dec 15.
Typ publikacji:
Journal Article
MeSH Terms:
Huntington Disease*/metabolism
Huntington Disease*/pathology
Animals ; Mice ; Rats ; Huntingtin Protein/genetics ; Huntingtin Protein/metabolism ; Molecular Chaperones/metabolism ; Proteomics ; Ubiquitin/metabolism ; Ubiquitination
Czasopismo naukowe
Tytuł:
The ubiquitin thioesterase YOD1 ameliorates mutant Huntingtin induced pathology in Drosophila.
Autorzy:
Farkas A; Department of Biochemistry and Molecular Biology, Faculty of Science and Informatics, University of Szeged, Közép Fasor 52, 6726, Szeged, Hungary.; Doctoral School in Biology, Faculty of Science and Informatics, University of Szeged, 6726, Szeged, Hungary.
Zsindely N; Department of Biochemistry and Molecular Biology, Faculty of Science and Informatics, University of Szeged, Közép Fasor 52, 6726, Szeged, Hungary.; Department of Genetics, Faculty of Science and Informatics, University of Szeged, Közép Fasor 52, 6726, Szeged, Hungary.
Nagy G; Department of Biochemistry and Molecular Biology, Faculty of Science and Informatics, University of Szeged, Közép Fasor 52, 6726, Szeged, Hungary.
Kovács L; Department of Genetics, Faculty of Science and Informatics, University of Szeged, Közép Fasor 52, 6726, Szeged, Hungary.; Divison of Biology and Biological Engineering, California Institute of Technology, 1200 East California Boulevard, Pasadena, 91125, USA.
Deák P; Department of Genetics, Faculty of Science and Informatics, University of Szeged, Közép Fasor 52, 6726, Szeged, Hungary.
Bodai L; Department of Biochemistry and Molecular Biology, Faculty of Science and Informatics, University of Szeged, Közép Fasor 52, 6726, Szeged, Hungary. .
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Źródło:
Scientific reports [Sci Rep] 2023 Dec 11; Vol. 13 (1), pp. 21951. Date of Electronic Publication: 2023 Dec 11.
Typ publikacji:
Journal Article
MeSH Terms:
Drosophila*/genetics
Drosophila*/metabolism
Huntington Disease*/metabolism
Animals ; Amyloid beta-Peptides/genetics ; Deubiquitinating Enzymes/genetics ; Disease Models, Animal ; Huntingtin Protein/genetics ; Huntingtin Protein/metabolism ; Mutation ; Ubiquitin/genetics
Czasopismo naukowe
Tytuł:
Charge within Nt17 peptides modulates huntingtin aggregation and initial lipid binding events.
Autorzy:
Stonebraker AR; The C. Eugene Bennett Department of Chemistry, West Virginia University, 217 Clark Hall, Morgantown, WV 26506, USA.
Hankin R; The C. Eugene Bennett Department of Chemistry, West Virginia University, 217 Clark Hall, Morgantown, WV 26506, USA.
Kapp KL; The C. Eugene Bennett Department of Chemistry, West Virginia University, 217 Clark Hall, Morgantown, WV 26506, USA.
Li P; The C. Eugene Bennett Department of Chemistry, West Virginia University, 217 Clark Hall, Morgantown, WV 26506, USA.
Valentine SJ; The C. Eugene Bennett Department of Chemistry, West Virginia University, 217 Clark Hall, Morgantown, WV 26506, USA.
Legleiter J; The C. Eugene Bennett Department of Chemistry, West Virginia University, 217 Clark Hall, Morgantown, WV 26506, USA; Rockefeller Neurosciences Institutes, West Virginia University, 1 Medical Center Dr., P.O. Box 9303, Morgantown, WV 26505, USA; Department of Neuroscience, West Virginia University, 1 Medical Center Dr., P.O. Box 9303, Morgantown, WV 26505, USA. Electronic address: .
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Źródło:
Biophysical chemistry [Biophys Chem] 2023 Dec; Vol. 303, pp. 107123. Date of Electronic Publication: 2023 Oct 12.
Typ publikacji:
Journal Article; Research Support, N.I.H., Extramural
MeSH Terms:
Peptides*/chemistry
Serine*/metabolism
Huntingtin Protein/genetics ; Huntingtin Protein/chemistry ; Huntingtin Protein/metabolism ; Phosphorylation ; Lipids
Czasopismo naukowe
Tytuł:
Mutant huntingtin confers cell-autonomous phenotypes on Huntington's disease iPSC-derived microglia.
Autorzy:
Stöberl N; School of Biosciences, Cardiff University, Cardiff, UK. .
Donaldson J; Centre for Neuropsychiatric Genetics and Genomics, Division of Psychological Medicine and Clinical Neurosciences, Cardiff University, Cardiff, UK.
Binda CS; Centre for Neuropsychiatric Genetics and Genomics, Division of Psychological Medicine and Clinical Neurosciences, Cardiff University, Cardiff, UK.
McAllister B; Centre for Neuropsychiatric Genetics and Genomics, Division of Psychological Medicine and Clinical Neurosciences, Cardiff University, Cardiff, UK.
Hall-Roberts H; UK Dementia Research Institute at Cardiff, Cardiff University, Cardiff, UK.
Jones L; Centre for Neuropsychiatric Genetics and Genomics, Division of Psychological Medicine and Clinical Neurosciences, Cardiff University, Cardiff, UK.
Massey TH; Centre for Neuropsychiatric Genetics and Genomics, Division of Psychological Medicine and Clinical Neurosciences, Cardiff University, Cardiff, UK.
Allen ND; School of Biosciences, Cardiff University, Cardiff, UK. .
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Źródło:
Scientific reports [Sci Rep] 2023 Nov 22; Vol. 13 (1), pp. 20477. Date of Electronic Publication: 2023 Nov 22.
Typ publikacji:
Journal Article
MeSH Terms:
Huntington Disease*/metabolism
Induced Pluripotent Stem Cells*
Neurodegenerative Diseases*/metabolism
Humans ; Huntingtin Protein/genetics ; Huntingtin Protein/metabolism ; Microglia/metabolism ; Phenotype
Czasopismo naukowe

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