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


Tytuł:
The polyglutamine domain is the primary driver of seeding in huntingtin aggregation.
Autorzy:
Skeens A; The C. Eugene Bennett Department of Chemistry, West Virginia University, Morgantown, West Virginia, United States of America.
Siriwardhana C; The C. Eugene Bennett Department of Chemistry, West Virginia University, Morgantown, West Virginia, United States of America.
Massinople SE; The C. Eugene Bennett Department of Chemistry, West Virginia University, Morgantown, West Virginia, United States of America.
Wunder MM; The C. Eugene Bennett Department of Chemistry, West Virginia University, Morgantown, West Virginia, United States of America.
Ellis ZL; The C. Eugene Bennett Department of Chemistry, West Virginia University, Morgantown, West Virginia, United States of America.
Keith KM; The C. Eugene Bennett Department of Chemistry, West Virginia University, Morgantown, West Virginia, United States of America.
Girman T; The C. Eugene Bennett Department of Chemistry, West Virginia University, Morgantown, West Virginia, United States of America.
Frey SL; The Department of Chemistry, Gettysburg College, Gettysburg, Pennsylvania, United States of America.
Legleiter J; The C. Eugene Bennett Department of Chemistry, West Virginia University, Morgantown, West Virginia, United States of America.; Rockefeller Neurosciences Institutes, West Virginia University, Morgantown, West Virginia, United States of America.; Department of Neuroscience, West Virginia University, Morgantown, West Virginia, United States of America.
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Źródło:
PloS one [PLoS One] 2024 Mar 14; Vol. 19 (3), pp. e0298323. Date of Electronic Publication: 2024 Mar 14 (Print Publication: 2024).
Typ publikacji:
Journal Article
MeSH Terms:
Neurodegenerative Diseases*
Huntington Disease*/genetics
Huntington Disease*/metabolism
Peptides*
Animals ; Humans ; Huntingtin Protein/genetics ; Caenorhabditis elegans/genetics ; Caenorhabditis elegans/metabolism ; Amyloid/metabolism ; Lipids
Czasopismo naukowe
Tytuł:
Huntingtin structure is orchestrated by HAP40 and shows a polyglutamine expansion-specific interaction with exon 1.
Autorzy:
Harding RJ; Structural Genomics Consortium, University of Toronto, Toronto, ON, M5G 1L7, Canada. .
Deme JC; Sir William Dunn School of Pathology, University of Oxford, South Parks Road, Oxford, OX1 3RE, UK.; Central Oxford Structural Molecular Imaging Centre, University of Oxford, South Parks Road, Oxford, OX1 3RE, UK.; Center for Structural Biology, Center for Cancer Research, National Cancer Institute, Frederick, MD, 21702, USA.
Hevler JF; Biomolecular Mass Spectrometry and Proteomics, Bijvoet Center for Biomolecular Research and Utrecht Institute of Pharmaceutical Sciences, Utrecht University, Padualaan 8, 3584 CH, Utrecht, The Netherlands.; Netherlands Proteomics Center, Padualaan 8, 3584 CH, Utrecht, The Netherlands.
Tamara S; Biomolecular Mass Spectrometry and Proteomics, Bijvoet Center for Biomolecular Research and Utrecht Institute of Pharmaceutical Sciences, Utrecht University, Padualaan 8, 3584 CH, Utrecht, The Netherlands.; Netherlands Proteomics Center, Padualaan 8, 3584 CH, Utrecht, The Netherlands.
Lemak A; Princess Margaret Cancer Centre and Department of Medical Biophysics, University of Toronto, Toronto, ON, M5G 1L7, Canada.
Cantle JP; Behavioral Neuroscience Program, Department of Psychology, Western Washington University, Bellingham, WA, 98225, USA.
Szewczyk MM; Structural Genomics Consortium, University of Toronto, Toronto, ON, M5G 1L7, Canada.
Begeja N; Department of Biochemistry and Biomedical Sciences, McMaster University, Hamilton, ON, L8S 4K1, Canada.
Goss S; Department of Biochemistry and Biomedical Sciences, McMaster University, Hamilton, ON, L8S 4K1, Canada.
Zuo X; X-ray Science Division, Argonne National Laboratory, Lemont, IL, 60439, USA.
Loppnau P; Structural Genomics Consortium, University of Toronto, Toronto, ON, M5G 1L7, Canada.
Seitova A; Structural Genomics Consortium, University of Toronto, Toronto, ON, M5G 1L7, Canada.
Hutchinson A; Structural Genomics Consortium, University of Toronto, Toronto, ON, M5G 1L7, Canada.
Fan L; Basic Science Program, Frederick National Laboratory for Cancer Research, SAXS Core of NCI, National Institutes of Health, Frederick, MD, 21701, USA.
Truant R; Department of Biochemistry and Biomedical Sciences, McMaster University, Hamilton, ON, L8S 4K1, Canada.
Schapira M; Structural Genomics Consortium, University of Toronto, Toronto, ON, M5G 1L7, Canada.; Department of Pharmacology and Toxicology, University of Toronto, Toronto, ON, M5S 1A8, Canada.
Carroll JB; Behavioral Neuroscience Program, Department of Psychology, Western Washington University, Bellingham, WA, 98225, USA.
Heck AJR; Biomolecular Mass Spectrometry and Proteomics, Bijvoet Center for Biomolecular Research and Utrecht Institute of Pharmaceutical Sciences, Utrecht University, Padualaan 8, 3584 CH, Utrecht, The Netherlands.; Netherlands Proteomics Center, Padualaan 8, 3584 CH, Utrecht, The Netherlands.
Lea SM; Sir William Dunn School of Pathology, University of Oxford, South Parks Road, Oxford, OX1 3RE, UK.; Central Oxford Structural Molecular Imaging Centre, University of Oxford, South Parks Road, Oxford, OX1 3RE, UK.; 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. .
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Źródło:
Communications biology [Commun Biol] 2021 Dec 08; Vol. 4 (1), pp. 1374. Date of Electronic Publication: 2021 Dec 08.
Typ publikacji:
Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.
MeSH Terms:
Exons*
Huntingtin Protein/*genetics
Huntington Disease/*genetics
Nuclear Proteins/*genetics
Peptides/*metabolism
Cryoelectron Microscopy ; Humans ; Huntingtin Protein/metabolism ; Huntingtin Protein/ultrastructure ; Nuclear Proteins/metabolism ; Nuclear Proteins/ultrastructure
Czasopismo naukowe
Tytuł:
Cryo-electron tomography provides topological insights into mutant huntingtin exon 1 and polyQ aggregates.
Autorzy:
Galaz-Montoya JG; Department of Bioengineering and James H. Clark Center, Stanford University, Stanford, CA, USA. .
Shahmoradian SH; Department of Biology and Chemistry, Laboratory of Biomolecular Research, Paul Scherrer Institute, Villigen, Switzerland.
Shen K; Department of Biology, Stanford University, Stanford, CA, USA.; Department of Molecular and Cell biology, University of California, Berkeley, CA, USA.
Frydman J; Department of Biology, Stanford University, Stanford, CA, USA.
Chiu W; Department of Bioengineering and James H. Clark Center, Stanford University, Stanford, CA, USA. .; Division of CryoEM and Bioimaging, SSRL, SLAC National Accelerator Laboratory, Menlo Park, CA, USA. .
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Źródło:
Communications biology [Commun Biol] 2021 Jul 08; Vol. 4 (1), pp. 849. Date of Electronic Publication: 2021 Jul 08.
Typ publikacji:
Journal Article; Research Support, N.I.H., Extramural
MeSH Terms:
Mutation*
Electron Microscope Tomography/*methods
Exons/*genetics
Huntingtin Protein/*genetics
Peptides/*genetics
Humans ; Huntingtin Protein/chemistry ; Huntingtin Protein/ultrastructure ; Huntington Disease/genetics ; Mutant Proteins/chemistry ; Mutant Proteins/genetics ; Mutant Proteins/ultrastructure ; Protein Aggregates ; Protein Aggregation, Pathological ; Protein Conformation
Czasopismo naukowe
Tytuł:
Membrane Interactions Accelerate the Self-Aggregation of Huntingtin Exon 1 Fragments in a Polyglutamine Length-Dependent Manner.
Autorzy:
Marquette A; Chemistry Institute UMR7177, University of Strasbourg/CNRS, 67000 Strasbourg, France.
Aisenbrey C; Chemistry Institute UMR7177, University of Strasbourg/CNRS, 67000 Strasbourg, France.
Bechinger B; Chemistry Institute UMR7177, University of Strasbourg/CNRS, 67000 Strasbourg, France.; Insitut Universitaire de France, 75005 Paris, France.
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Źródło:
International journal of molecular sciences [Int J Mol Sci] 2021 Jun 23; Vol. 22 (13). Date of Electronic Publication: 2021 Jun 23.
Typ publikacji:
Journal Article
MeSH Terms:
Cell Membrane/*metabolism
Huntingtin Protein/*metabolism
Peptides/*metabolism
Benzothiazoles/chemistry ; Circular Dichroism ; Dynamic Light Scattering ; Exons ; Fluorescence ; Humans ; Huntingtin Protein/chemistry ; Huntingtin Protein/genetics ; Lipid Bilayers ; Peptide Fragments/genetics ; Peptide Fragments/metabolism ; Peptides/chemistry
Czasopismo naukowe
Tytuł:
PolyQ-expanded proteins impair cellular proteostasis of ataxin-3 through sequestering the co-chaperone HSJ1 into aggregates.
Autorzy:
Yue HW; State Key Laboratory of Molecular Biology, Shanghai Institute of Biochemistry and Cell Biology, Center for Excellence in Molecular Cell Science, Chinese Academy of Sciences, Shanghai, 200031, People's Republic of China.; University of Chinese Academy of Sciences, Beijing, 100049, People's Republic of China.
Hong JY; State Key Laboratory of Molecular Biology, Shanghai Institute of Biochemistry and Cell Biology, Center for Excellence in Molecular Cell Science, Chinese Academy of Sciences, Shanghai, 200031, People's Republic of China.; University of Chinese Academy of Sciences, Beijing, 100049, People's Republic of China.
Zhang SX; State Key Laboratory of Molecular Biology, Shanghai Institute of Biochemistry and Cell Biology, Center for Excellence in Molecular Cell Science, Chinese Academy of Sciences, Shanghai, 200031, People's Republic of China.; University of Chinese Academy of Sciences, Beijing, 100049, People's Republic of China.
Jiang LL; State Key Laboratory of Molecular Biology, Shanghai Institute of Biochemistry and Cell Biology, Center for Excellence in Molecular Cell Science, Chinese Academy of Sciences, Shanghai, 200031, People's Republic of China.
Hu HY; State Key Laboratory of Molecular Biology, Shanghai Institute of Biochemistry and Cell Biology, Center for Excellence in Molecular Cell Science, Chinese Academy of Sciences, Shanghai, 200031, People's Republic of China. .
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Źródło:
Scientific reports [Sci Rep] 2021 Apr 09; Vol. 11 (1), pp. 7815. Date of Electronic Publication: 2021 Apr 09.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Ataxin-3/*metabolism
HSP40 Heat-Shock Proteins/*metabolism
Molecular Chaperones/*metabolism
Neurodegenerative Diseases/*metabolism
Peptides/*metabolism
Protein Aggregates/*genetics
Protein Aggregation, Pathological/*metabolism
Proteostasis/*genetics
Repressor Proteins/*metabolism
Amyloid/metabolism ; Amyloidogenic Proteins/metabolism ; Ataxin-3/chemistry ; Ataxin-3/genetics ; HEK293 Cells ; Humans ; Huntingtin Protein/metabolism ; Inclusion Bodies/metabolism ; Intracellular Space/metabolism ; Proteasome Endopeptidase Complex/metabolism ; Protein Aggregation, Pathological/genetics ; Protein Domains/genetics ; Proteolysis ; Repressor Proteins/chemistry ; Repressor Proteins/genetics ; Signal Transduction/genetics ; Solubility ; Transfection
Czasopismo naukowe
Tytuł:
SGTA associates with intracellular aggregates in neurodegenerative diseases.
Autorzy:
Kubota S; Department of Neurology and Stroke Medicine, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan.
Doi H; Department of Neurology and Stroke Medicine, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan. .
Koyano S; Department of Neurology and Stroke Medicine, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan.; Department of Neurology, Yokohama Minami Kyosai Hospital, 1-21-1 Mutuurahigashi, Kanazawa-ku, Yokohama, 236-0037, Japan.
Tanaka K; Department of Neurology and Stroke Medicine, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan.
Komiya H; Department of Neurology and Stroke Medicine, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan.
Katsumoto A; Department of Neurology and Stroke Medicine, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan.
Ikeda S; Department of Neurology and Stroke Medicine, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan.
Hashiguchi S; Department of Neurology and Stroke Medicine, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan.
Nakamura H; Department of Neurology and Stroke Medicine, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan.
Fukai R; Department of Neurology and Stroke Medicine, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan.
Takahashi K; Department of Neurology and Stroke Medicine, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan.
Kunii M; Department of Neurology and Stroke Medicine, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan.
Tada M; Department of Neurology and Stroke Medicine, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan.
Takeuchi H; Department of Neurology and Stroke Medicine, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan.
Tanaka F; Department of Neurology and Stroke Medicine, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan. .
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Źródło:
Molecular brain [Mol Brain] 2021 Mar 23; Vol. 14 (1), pp. 59. Date of Electronic Publication: 2021 Mar 23.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Brain Chemistry*
Protein Aggregates*
Molecular Chaperones/*metabolism
Nerve Tissue Proteins/*metabolism
Neurodegenerative Diseases/*metabolism
Peptides/*metabolism
Protein Aggregation, Pathological/*metabolism
Animals ; Autopsy ; Brain/pathology ; Cell Line, Tumor ; Humans ; Huntingtin Protein/genetics ; Huntingtin Protein/metabolism ; Inclusion Bodies/chemistry ; Mice ; Mice, Transgenic ; Neuroblastoma ; Neurodegenerative Diseases/pathology ; Peptide Fragments/genetics ; Peptide Fragments/metabolism ; Recombinant Proteins/metabolism ; Solubility ; Subcellular Fractions/metabolism ; Transfection ; alpha-Synuclein/analysis
Czasopismo naukowe
Tytuł:
Meso scale discovery-based assays for the detection of aggregated huntingtin.
Autorzy:
Reindl W; Evotec AG, Hamburg, Germany.
Baldo B; Evotec AG, Hamburg, Germany.
Schulz J; Evotec AG, Hamburg, Germany.
Janack I; Evotec AG, Hamburg, Germany.
Lindner I; Evotec AG, Hamburg, Germany.
Kleinschmidt M; Evotec AG, Hamburg, Germany.
Sedaghat Y; Evotec AG, Hamburg, Germany.
Thiede C; Evotec AG, Hamburg, Germany.
Tillack K; Evotec AG, Hamburg, Germany.
Schmidt C; Evotec AG, Hamburg, Germany.
Cardaun I; Evotec AG, Hamburg, Germany.
Schwagarus T; Evotec AG, Hamburg, Germany.
Herrmann F; Evotec AG, Hamburg, Germany.
Hotze M; Evotec AG, Hamburg, Germany.
Osborne GF; Dept. Neurodegenerative Disease, Huntington's Disease Centre and Dementia Research Institute, Institute of Neurology, University College London, London, United Kingdom.
Herrmann S; Evotec AG, Hamburg, Germany.
Weiss A; Evotec AG, Hamburg, Germany.
Zerbinatti C; Evotec AG, Hamburg, Germany.
Bates GP; Dept. Neurodegenerative Disease, Huntington's Disease Centre and Dementia Research Institute, Institute of Neurology, University College London, London, United Kingdom.
Bard J; CHDI Management/CHDI Foundation, Los Angeles, CA, United States of America.
Munoz-Sanjuan I; CHDI Management/CHDI Foundation, Los Angeles, CA, United States of America.
Macdonald D; CHDI Management/CHDI Foundation, Los Angeles, CA, United States of America.
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Źródło:
PloS one [PLoS One] 2019 Mar 26; Vol. 14 (3), pp. e0213521. Date of Electronic Publication: 2019 Mar 26 (Print Publication: 2019).
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Biological Assay*
Brain/*metabolism
Huntingtin Protein/*metabolism
Huntington Disease/*metabolism
Peptides/*metabolism
Protein Aggregation, Pathological/*metabolism
Animals ; Disease Models, Animal ; Humans ; Huntingtin Protein/genetics ; Huntington Disease/genetics ; Huntington Disease/pathology ; Mice ; Mice, Transgenic ; Peptides/genetics ; Protein Aggregation, Pathological/genetics ; Protein Aggregation, Pathological/pathology
Czasopismo naukowe
Tytuł:
Immiscible inclusion bodies formed by polyglutamine and poly(glycine-alanine) are enriched with distinct proteomes but converge in proteins that are risk factors for disease and involved in protein degradation.
Autorzy:
Radwan M; Department of Biochemistry and Molecular Biology; and Bio21 Molecular Science and Biotechnology Institute, The University of Melbourne, Parkville, VIC, Australia.
Lilley JD; Department of Biochemistry and Molecular Biology; and Bio21 Molecular Science and Biotechnology Institute, The University of Melbourne, Parkville, VIC, Australia.
Ang CS; Bio21 Mass Spectrometry and Proteomics Facility, The University of Melbourne, Parkville, Victoria, Australia.
Reid GE; Department of Biochemistry and Molecular Biology; and Bio21 Molecular Science and Biotechnology Institute, The University of Melbourne, Parkville, VIC, Australia.; School of Chemistry, The University of Melbourne, Parkville, VIC, Australia.
Hatters DM; Department of Biochemistry and Molecular Biology; and Bio21 Molecular Science and Biotechnology Institute, The University of Melbourne, Parkville, VIC, Australia.
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Źródło:
PloS one [PLoS One] 2020 Aug 28; Vol. 15 (8), pp. e0233247. Date of Electronic Publication: 2020 Aug 28 (Print Publication: 2020).
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Inclusion Bodies/*metabolism
Peptides/*metabolism
Proteins/*metabolism
Animals ; C9orf72 Protein/genetics ; C9orf72 Protein/metabolism ; Cell Line ; Exons ; Humans ; Huntingtin Protein/genetics ; Huntingtin Protein/metabolism ; Inclusion Bodies/genetics ; Inclusion Bodies/pathology ; Luminescent Proteins/genetics ; Luminescent Proteins/metabolism ; Mice ; Microscopy, Confocal ; Mutation ; Nerve Tissue Proteins/genetics ; Nerve Tissue Proteins/metabolism ; Neurodegenerative Diseases/genetics ; Neurodegenerative Diseases/metabolism ; Neurodegenerative Diseases/pathology ; Peptides/genetics ; Protein Aggregation, Pathological/genetics ; Protein Aggregation, Pathological/metabolism ; Protein Aggregation, Pathological/pathology ; Proteins/genetics ; Proteolysis ; Proteome/genetics ; Proteome/metabolism ; Recombinant Fusion Proteins/genetics ; Recombinant Fusion Proteins/metabolism ; Risk Factors ; Solubility
Czasopismo naukowe
Tytuł:
Co-chaperones DNAJA1 and DNAJB6 are critical for regulation of polyglutamine aggregation.
Autorzy:
Rodríguez-González C; Molecular Neurobiology, Department of Experimental Medical Science, Lund University, BMC B11, 22184, Lund, Sweden.
Lin S; Molecular Neurobiology, Department of Experimental Medical Science, Lund University, BMC B11, 22184, Lund, Sweden.
Arkan S; Molecular Neurobiology, Department of Experimental Medical Science, Lund University, BMC B11, 22184, Lund, Sweden.
Hansen C; Molecular Neurobiology, Department of Experimental Medical Science, Lund University, BMC B11, 22184, Lund, Sweden. .
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Źródło:
Scientific reports [Sci Rep] 2020 May 18; Vol. 10 (1), pp. 8130. Date of Electronic Publication: 2020 May 18.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
HSP40 Heat-Shock Proteins/*metabolism
Molecular Chaperones/*metabolism
Nerve Tissue Proteins/*metabolism
Peptides/*chemistry
Peptides/*metabolism
HEK293 Cells ; HSP40 Heat-Shock Proteins/genetics ; Humans ; Huntingtin Protein/chemistry ; Huntingtin Protein/genetics ; Huntingtin Protein/metabolism ; Huntington Disease/genetics ; Huntington Disease/metabolism ; Molecular Chaperones/genetics ; Nerve Tissue Proteins/genetics ; Protein Aggregates
Czasopismo naukowe
Tytuł:
Frequency and distribution of polyQ disease intermediate-length repeat alleles in healthy Italian population.
Autorzy:
Mongelli A; Unit of Medical Genetics and Neurogenetics, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
Magri S; Unit of Medical Genetics and Neurogenetics, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
Salvatore E; Department of Neurosciences and Reproductive and Odontostomatological Sciences, Federico II University, Naples, Italy.
Rizzo E; Unit of Medical Genetics and Neurogenetics, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
De Rosa A; Department of Neurosciences and Reproductive and Odontostomatological Sciences, Federico II University, Naples, Italy.
Fico T; Department of Neurosciences and Reproductive and Odontostomatological Sciences, Federico II University, Naples, Italy.
Gatti M; Unit of Medical Genetics and Neurogenetics, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
Gellera C; Unit of Medical Genetics and Neurogenetics, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
Taroni F; Unit of Medical Genetics and Neurogenetics, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
Mariotti C; Unit of Medical Genetics and Neurogenetics, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy. .
Nanetti L; Unit of Medical Genetics and Neurogenetics, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
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Źródło:
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology [Neurol Sci] 2020 Jun; Vol. 41 (6), pp. 1475-1482. Date of Electronic Publication: 2020 Jan 15.
Typ publikacji:
Journal Article
MeSH Terms:
Gene Frequency/*genetics
Huntington Disease/*genetics
Peptides/*genetics
Spinocerebellar Ataxias/*genetics
Trinucleotide Repeats/*genetics
Adult ; Aged ; Alleles ; Ataxin-1/genetics ; Ataxin-2/genetics ; Female ; Humans ; Huntingtin Protein/genetics ; Huntington Disease/epidemiology ; Italy/epidemiology ; Male ; Middle Aged ; Prevalence ; Spinocerebellar Ataxias/epidemiology ; TATA-Box Binding Protein/genetics
Czasopismo naukowe
Tytuł:
N-terminal Huntingtin Knock-In Mice: Implications of Removing the N-terminal Region of Huntingtin for Therapy.
Autorzy:
Liu X; State Key Laboratory of Molecular Developmental Biology, Institute of Genetics and Developmental Biology, Chinese Academy of Sciences, Beijing, China.; Department of Human Genetics, Emory University School of Medicine, Atlanta, Georgia, United States of America.; Graduate School of the Chinese Academy of Sciences, Beijing, China.
Wang CE; Department of Human Genetics, Emory University School of Medicine, Atlanta, Georgia, United States of America.
Hong Y; Department of Human Genetics, Emory University School of Medicine, Atlanta, Georgia, United States of America.
Zhao T; Department of Human Genetics, Emory University School of Medicine, Atlanta, Georgia, United States of America.
Wang G; State Key Laboratory of Molecular Developmental Biology, Institute of Genetics and Developmental Biology, Chinese Academy of Sciences, Beijing, China.; Department of Human Genetics, Emory University School of Medicine, Atlanta, Georgia, United States of America.; Graduate School of the Chinese Academy of Sciences, Beijing, China.
Gaertig MA; Department of Human Genetics, Emory University School of Medicine, Atlanta, Georgia, United States of America.
Sun M; Department of Human Genetics, Emory University School of Medicine, Atlanta, Georgia, United States of America.
Li S; Department of Human Genetics, Emory University School of Medicine, Atlanta, Georgia, United States of America.
Li XJ; State Key Laboratory of Molecular Developmental Biology, Institute of Genetics and Developmental Biology, Chinese Academy of Sciences, Beijing, China.; Department of Human Genetics, Emory University School of Medicine, Atlanta, Georgia, United States of America.
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Źródło:
PLoS genetics [PLoS Genet] 2016 May 20; Vol. 12 (5), pp. e1006083. Date of Electronic Publication: 2016 May 20 (Print Publication: 2016).
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't; Research Support, N.I.H., Extramural
MeSH Terms:
Huntingtin Protein/*genetics
Huntington Disease/*genetics
Nerve Degeneration/*genetics
Nerve Tissue Proteins/*genetics
Peptides/*genetics
Animals ; Corpus Striatum/metabolism ; Corpus Striatum/pathology ; Gene Knock-In Techniques ; Humans ; Huntingtin Protein/metabolism ; Huntingtin Protein/therapeutic use ; Huntington Disease/pathology ; Huntington Disease/therapy ; Mice ; Mutation ; Nerve Degeneration/pathology ; Nerve Degeneration/therapy ; Nerve Tissue Proteins/metabolism ; Nerve Tissue Proteins/therapeutic use ; Neurons/metabolism ; Neurons/pathology ; Peptides/metabolism ; Peptides/therapeutic use ; Phenotype
Czasopismo naukowe
Tytuł:
N-Terminal Fragments of Huntingtin Longer than Residue 170 form Visible Aggregates Independently to Polyglutamine Expansion.
Autorzy:
Chen MZ; Department of Biochemistry and Molecular Biology and Bio21 Molecular Science and Biotechnology Institute, The University of Melbourne, VIC, Australia.
Mok SA; Department of Neurology, University of California San Francisco, San Francisco, CA, USA.
Ormsby AR; Department of Biochemistry and Molecular Biology and Bio21 Molecular Science and Biotechnology Institute, The University of Melbourne, VIC, Australia.
Muchowski PJ; KynuRex, 634 Los Palmos Drive, San Francisco, CA, USA.
Hatters DM; Department of Biochemistry and Molecular Biology and Bio21 Molecular Science and Biotechnology Institute, The University of Melbourne, VIC, Australia.
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Źródło:
Journal of Huntington's disease [J Huntingtons Dis] 2017; Vol. 6 (1), pp. 79-91.
Typ publikacji:
Journal Article
MeSH Terms:
DNA Repeat Expansion*
Peptides*/genetics
Peptides*/metabolism
Huntingtin Protein/*metabolism
Protein Aggregation, Pathological/*metabolism
Animals ; Blotting, Western ; COS Cells ; Cell Line, Tumor ; Chlorocebus aethiops ; Flow Cytometry ; Fluorescent Antibody Technique ; Genetic Vectors ; HEK293 Cells ; Humans ; Huntingtin Protein/genetics ; Inclusion Bodies/metabolism ; Inclusion Bodies/pathology ; Luminescent Proteins/genetics ; Luminescent Proteins/metabolism ; Mice ; Microscopy, Confocal ; Protein Aggregation, Pathological/genetics ; Protein Aggregation, Pathological/pathology ; Transfection ; Red Fluorescent Protein
Czasopismo naukowe
Tytuł:
Multiple discrete soluble aggregates influence polyglutamine toxicity in a Huntington's disease model system.
Autorzy:
Xi W; Department of Molecular, Cellular, and Biomedical Sciences, Rudman Hall, University of New Hampshire, NH 03824, Durham, USA.
Wang X; Department of Molecular, Cellular, and Biomedical Sciences, Rudman Hall, University of New Hampshire, NH 03824, Durham, USA.
Laue TM; Department of Molecular, Cellular, and Biomedical Sciences, Rudman Hall, University of New Hampshire, NH 03824, Durham, USA.
Denis CL; Department of Molecular, Cellular, and Biomedical Sciences, Rudman Hall, University of New Hampshire, NH 03824, Durham, USA.
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Źródło:
Scientific reports [Sci Rep] 2016 Oct 10; Vol. 6, pp. 34916. Date of Electronic Publication: 2016 Oct 10.
Typ publikacji:
Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.
MeSH Terms:
Protein Aggregates*
Huntingtin Protein/*metabolism
Huntington Disease/*physiopathology
Peptides/*toxicity
Humans ; Huntingtin Protein/genetics ; Models, Biological ; Peptides/genetics ; Ultracentrifugation ; Yeasts/genetics ; Yeasts/metabolism
Czasopismo naukowe
Tytuł:
Nanoscale studies link amyloid maturity with polyglutamine diseases onset.
Autorzy:
Ruggeri FS; Laboratory of Physics of Living Matter, École Polytechnique Fédérale de Lausanne (EPFL), 1015 Lausanne, Switzerland.; Department of Chemistry, University of Cambridge, CB21EW, United Kingdom.
Vieweg S; Laboratory of Molecular and Chemical Biology of Neurodegeneration, Brain Mind Institute, École Polytechnique Fédérale de Lausanne (EPFL), 1015 Lausanne, Switzerland.
Cendrowska U; Laboratory of Physics of Living Matter, École Polytechnique Fédérale de Lausanne (EPFL), 1015 Lausanne, Switzerland.; Laboratory of Molecular and Chemical Biology of Neurodegeneration, Brain Mind Institute, École Polytechnique Fédérale de Lausanne (EPFL), 1015 Lausanne, Switzerland.
Longo G; Laboratory of Physics of Living Matter, École Polytechnique Fédérale de Lausanne (EPFL), 1015 Lausanne, Switzerland.
Chiki A; Laboratory of Molecular and Chemical Biology of Neurodegeneration, Brain Mind Institute, École Polytechnique Fédérale de Lausanne (EPFL), 1015 Lausanne, Switzerland.
Lashuel HA; Laboratory of Molecular and Chemical Biology of Neurodegeneration, Brain Mind Institute, École Polytechnique Fédérale de Lausanne (EPFL), 1015 Lausanne, Switzerland.; Qatar Biomedical Research Institute (QBRI), Hamad Bin Kahlifa University (HBKU), P.O Box 5825, Doha, Qatar.
Dietler G; Laboratory of Physics of Living Matter, École Polytechnique Fédérale de Lausanne (EPFL), 1015 Lausanne, Switzerland.
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Źródło:
Scientific reports [Sci Rep] 2016 Aug 08; Vol. 6, pp. 31155. Date of Electronic Publication: 2016 Aug 08.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Huntington Disease*
Protein Aggregation, Pathological*
Amyloid/*chemistry
Huntingtin Protein/*chemistry
Peptides/*chemistry
Amyloid/metabolism ; Humans ; Huntingtin Protein/metabolism ; Peptides/metabolism
Czasopismo naukowe
Tytuł:
A short peptide protects from age‐onset proteotoxicity.
Autorzy:
Elsana, Hassan (AUTHOR)
Bruck‐Haimson, Reut (AUTHOR)
Zhu, Huadong (AUTHOR)
Siddiqui, Atif Ahmed (AUTHOR)
Zaretsky, Adam (AUTHOR)
Cohen, Irit (AUTHOR)
Boocholez, Hana (AUTHOR)
Roitenberg, Noa (AUTHOR)
Moll, Lorna (AUTHOR)
Plaschkes, Inbar (AUTHOR)
Naor, David (AUTHOR)
Cohen, Ehud (AUTHOR)
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Źródło:
Aging Cell. Dec2023, Vol. 22 Issue 12, p1-16. 16p.
Czasopismo naukowe
Tytuł:
Evolving Notch polyQ tracts reveal possible solenoid interference elements.
Autorzy:
Erives AJ; Department of Biology University of Iowa Iowa City, IA, United States of America.
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Źródło:
PloS one [PLoS One] 2017 Mar 20; Vol. 12 (3), pp. e0174253. Date of Electronic Publication: 2017 Mar 20 (Print Publication: 2017).
Typ publikacji:
Journal Article
MeSH Terms:
Evolution, Molecular*
Peptides*/genetics
Repetitive Sequences, Nucleic Acid*
Drosophila Proteins/*genetics
Receptors, Notch/*genetics
Animals ; Drosophila Proteins/metabolism ; Drosophila melanogaster ; Huntingtin Protein/genetics ; Hydrogen Bonding ; Models, Genetic ; Models, Molecular ; Protein Domains ; Protein Folding ; Protein Structure, Secondary ; Receptors, Notch/metabolism ; Sequence Homology, Amino Acid
Czasopismo naukowe
Tytuł:
FUS/TLS acts as an aggregation-dependent modifier of polyglutamine disease model mice.
Autorzy:
Kino Y; CREST(Core Research for Evolutionary Science and Technology), JST, Saitama, Japan.; Department of Neuroscience for Neurodegenerative Disorders, Juntendo University Graduate School of Medicine, Tokyo, Japan.; Laboratory for Structural Neuropathology , Brain Science Institute, RIKEN, Saitama, Japan.; Laboratory for Molecular Mechanisms of Thalamus Development, Brain Science Institute, RIKEN, Saitama, Japan.; Department of Bioinformatics and Molecular Neuropathology, Meiji Pharmaceutical University, Tokyo, Japan.
Washizu C; Laboratory for Structural Neuropathology , Brain Science Institute, RIKEN, Saitama, Japan.
Kurosawa M; CREST(Core Research for Evolutionary Science and Technology), JST, Saitama, Japan.; Department of Neuroscience for Neurodegenerative Disorders, Juntendo University Graduate School of Medicine, Tokyo, Japan.; Laboratory for Structural Neuropathology , Brain Science Institute, RIKEN, Saitama, Japan.; Laboratory for Molecular Mechanisms of Thalamus Development, Brain Science Institute, RIKEN, Saitama, Japan.
Yamada M; Laboratory for Structural Neuropathology , Brain Science Institute, RIKEN, Saitama, Japan.
Doi H; Department of Clinical Neurology and Stroke Medicine, Graduate School of Medicine, Yokohama City University, Yokohama, Japan.
Takumi T; CREST(Core Research for Evolutionary Science and Technology), JST, Saitama, Japan.; Laboratory for Mental Biology, Brain Science Institute, RIKEN, Saitama, Japan.; Graduate School of Biomedical Sciences, Hiroshima University, Hiroshima, Japan.
Adachi H; Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Katsuno M; Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Sobue G; Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Hicks GG; Manitoba Institute of Cell Biology, University of Manitoba, Winnipeg, Canada.
Hattori N; Department of Neuroscience for Neurodegenerative Disorders, Juntendo University Graduate School of Medicine, Tokyo, Japan.
Shimogori T; Laboratory for Molecular Mechanisms of Thalamus Development, Brain Science Institute, RIKEN, Saitama, Japan.
Nukina N; CREST(Core Research for Evolutionary Science and Technology), JST, Saitama, Japan.; Department of Neuroscience for Neurodegenerative Disorders, Juntendo University Graduate School of Medicine, Tokyo, Japan.; Laboratory for Structural Neuropathology , Brain Science Institute, RIKEN, Saitama, Japan.; Laboratory for Molecular Mechanisms of Thalamus Development, Brain Science Institute, RIKEN, Saitama, Japan.; Laboratory of Structural Neuropathology, Doshisha University Graduate School of Brain Science, Kyoto, Japan.
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Źródło:
Scientific reports [Sci Rep] 2016 Oct 14; Vol. 6, pp. 35236. Date of Electronic Publication: 2016 Oct 14.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Neurodegenerative Diseases/*metabolism
Peptides/*metabolism
RNA-Binding Protein FUS/*metabolism
Animals ; DNA-Binding Proteins/metabolism ; Disease Models, Animal ; Female ; Homozygote ; Huntingtin Protein/metabolism ; Inclusion Bodies/metabolism ; Male ; Mice ; Mice, Inbred C57BL ; Mice, Knockout ; Mice, Transgenic/metabolism ; Mutation/physiology ; Phenotype ; RNA-Binding Proteins/metabolism ; Up-Regulation/physiology
Czasopismo naukowe
Tytuł:
Comparative analysis of anti-polyglutamine Fab crystals grown on Earth and in microgravity.
Autorzy:
Owens GE; Division of Biology and Biological Engineering, California Institute of Technology, 1200 East California Boulevard, Pasadena, CA 91125, USA.
New DM; Division of Biology and Biological Engineering, California Institute of Technology, 1200 East California Boulevard, Pasadena, CA 91125, USA.
Olvera AI; Division of Biology and Biological Engineering, California Institute of Technology, 1200 East California Boulevard, Pasadena, CA 91125, USA.
Manzella JA; Center for Biophysical Sciences and Engineering, University of Alabama at Birmingham, 1025 18th Street South, Birmingham, AL 35294, USA.
Macon BL; Center for Biophysical Sciences and Engineering, University of Alabama at Birmingham, 1025 18th Street South, Birmingham, AL 35294, USA.
Dunn JC; Center for Biophysical Sciences and Engineering, University of Alabama at Birmingham, 1025 18th Street South, Birmingham, AL 35294, USA.
Cooper DA; Center for Biophysical Sciences and Engineering, University of Alabama at Birmingham, 1025 18th Street South, Birmingham, AL 35294, USA.
Rouleau RL; Center for Biophysical Sciences and Engineering, University of Alabama at Birmingham, 1025 18th Street South, Birmingham, AL 35294, USA.
Connor DS; Center for Biophysical Sciences and Engineering, University of Alabama at Birmingham, 1025 18th Street South, Birmingham, AL 35294, USA.
Bjorkman PJ; Division of Biology and Biological Engineering, California Institute of Technology, 1200 East California Boulevard, Pasadena, CA 91125, USA.
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Źródło:
Acta crystallographica. Section F, Structural biology communications [Acta Crystallogr F Struct Biol Commun] 2016 Oct 01; Vol. 72 (Pt 10), pp. 762-771. Date of Electronic Publication: 2016 Sep 22.
Typ publikacji:
Comparative Study; Journal Article
MeSH Terms:
Weightlessness*
Immunoglobulin Fab Fragments/*chemistry
Peptides/*antagonists & inhibitors
Crystallization ; Crystallography, X-Ray ; Earth, Planet ; Gene Expression ; Humans ; Huntingtin Protein/chemistry ; Immunoglobulin Fab Fragments/biosynthesis ; Immunoglobulin Fab Fragments/genetics ; Peptides/chemistry ; Recombinant Proteins/chemistry ; Recombinant Proteins/genetics ; Recombinant Proteins/metabolism ; X-Ray Diffraction
Czasopismo naukowe
Tytuł:
Cytoplasmic Ubiquitin-Specific Protease 19 (USP19) Modulates Aggregation of Polyglutamine-Expanded Ataxin-3 and Huntingtin through the HSP90 Chaperone.
Autorzy:
He WT; State Key Laboratory of Molecular Biology, Institute of Biochemistry and Cell Biology, Shanghai Institutes for Biological Sciences, Chinese Academy of Sciences, Shanghai 200031, China.
Zheng XM; State Key Laboratory of Molecular Biology, Institute of Biochemistry and Cell Biology, Shanghai Institutes for Biological Sciences, Chinese Academy of Sciences, Shanghai 200031, China.; Department of Biochemistry and Molecular Biology, School of Medical Technology, Jiangsu University, Zhenjiang 212013, Jiangsu, China.
Zhang YH; State Key Laboratory of Molecular Biology, Institute of Biochemistry and Cell Biology, Shanghai Institutes for Biological Sciences, Chinese Academy of Sciences, Shanghai 200031, China.
Gao YG; State Key Laboratory of Molecular Biology, Institute of Biochemistry and Cell Biology, Shanghai Institutes for Biological Sciences, Chinese Academy of Sciences, Shanghai 200031, China.
Song AX; State Key Laboratory of Molecular Biology, Institute of Biochemistry and Cell Biology, Shanghai Institutes for Biological Sciences, Chinese Academy of Sciences, Shanghai 200031, China.
van der Goot FG; Global Health Institute, Ecole Polytechnique Fédérale de Lausanne (EPFL), Lausanne, Switzerland.
Hu HY; State Key Laboratory of Molecular Biology, Institute of Biochemistry and Cell Biology, Shanghai Institutes for Biological Sciences, Chinese Academy of Sciences, Shanghai 200031, China.
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Źródło:
PloS one [PLoS One] 2016 Jan 25; Vol. 11 (1), pp. e0147515. Date of Electronic Publication: 2016 Jan 25 (Print Publication: 2016).
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Ataxin-3/*metabolism
Cytoplasm/*enzymology
Endopeptidases/*metabolism
HSP90 Heat-Shock Proteins/*metabolism
Nerve Tissue Proteins/*metabolism
Peptides/*metabolism
Repressor Proteins/*metabolism
HEK293 Cells ; Humans ; Huntingtin Protein ; Up-Regulation
Czasopismo naukowe

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