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Tytuł:
Nationwide survey of patients with multisystem proteinopathy in Japan.
Autorzy:
Yamashita S; Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.; Department of Neurology, International University of Health and Welfare Narita Hospital, Narita, Japan.
Takahashi Y; Department of Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Tokyo, Japan.
Hashimoto J; Department of Orthopaedic Surgery, National Hospital Organization Osaka Minami Medical Center, Osaka, Japan.
Murakami A; Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Nakamura R; Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.; Department of Neurology, Aichi Medical University School of Medicine, Nagakute, Aichi, Japan.
Katsuno M; Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.; Department of Clinical Research Education, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Izumi R; Department of Neurology, Tohoku University Graduate School of Medicine, Sendai, Japan.
Suzuki N; Department of Neurology, Tohoku University Graduate School of Medicine, Sendai, Japan.
Warita H; Department of Neurology, Tohoku University Graduate School of Medicine, Sendai, Japan.
Aoki M; Department of Neurology, Tohoku University Graduate School of Medicine, Sendai, Japan.
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Corporate Authors:
Japan MSP Study Group
Źródło:
Annals of clinical and translational neurology [Ann Clin Transl Neurol] 2024 Apr; Vol. 11 (4), pp. 938-945. Date of Electronic Publication: 2024 Jan 29.
Typ publikacji:
Journal Article
MeSH Terms:
Frontotemporal Dementia*/genetics
Frontotemporal Dementia*/pathology
Muscular Diseases*
Motor Neuron Disease*
Parkinsonian Disorders*
Humans ; Japan/epidemiology ; Valosin Containing Protein/genetics ; RNA-Binding Proteins ; Nuclear Matrix-Associated Proteins
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Valosin containing protein (VCP): initiator, modifier, and potential drug target for neurodegenerative diseases.
Autorzy:
Chu S; Department of Physiology, McGill University, Montreal, HG3 1Y6, Canada.
Xie X; Department of Physiology, McGill University, Montreal, HG3 1Y6, Canada.
Payan C; Department of Physiology, McGill University, Montreal, HG3 1Y6, Canada.
Stochaj U; Department of Physiology, McGill University, Montreal, HG3 1Y6, Canada. .; Quantitative Life Sciences Program, McGill University, Montreal, Canada. .
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Źródło:
Molecular neurodegeneration [Mol Neurodegener] 2023 Aug 07; Vol. 18 (1), pp. 52. Date of Electronic Publication: 2023 Aug 07.
Typ publikacji:
Journal Article; Review; Research Support, Non-U.S. Gov't
MeSH Terms:
Neurodegenerative Diseases*/genetics
Humans ; Valosin Containing Protein/genetics ; Valosin Containing Protein/metabolism ; Adenosine Triphosphatases/genetics ; Adenosine Triphosphatases/metabolism ; Mutation ; Proteostasis ; Cell Cycle Proteins/genetics ; Cell Cycle Proteins/metabolism
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
In silico prediction, characterization, docking studies and molecular dynamics simulation of human p97 in complex with p37 cofactor.
Autorzy:
Mirzadeh A; Department of Biochemistry and Pharmacology, Bio21 Molecular Science and Biotechnology Institute, The University of Melbourne, Parkville, VIC, Australia.
Kobakhidze G; Department of Biochemistry and Pharmacology, Bio21 Molecular Science and Biotechnology Institute, The University of Melbourne, Parkville, VIC, Australia.
Vuillemot R; Department of Biochemistry and Pharmacology, Bio21 Molecular Science and Biotechnology Institute, The University of Melbourne, Parkville, VIC, Australia.; IMPMC - UMR 7590, CNRS, Sorbonne Université, Muséum National d'Histoire Naturelle, Paris, France.
Jonic S; IMPMC - UMR 7590, CNRS, Sorbonne Université, Muséum National d'Histoire Naturelle, Paris, France.
Rouiller I; Department of Biochemistry and Pharmacology, Bio21 Molecular Science and Biotechnology Institute, The University of Melbourne, Parkville, VIC, Australia. .
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Źródło:
BMC molecular and cell biology [BMC Mol Cell Biol] 2022 Sep 10; Vol. 23 (1), pp. 39. Date of Electronic Publication: 2022 Sep 10.
Typ publikacji:
Journal Article
MeSH Terms:
Adenosine Triphosphatases*/metabolism
Molecular Dynamics Simulation*
Adaptor Proteins, Signal Transducing/*chemistry
Valosin Containing Protein/*chemistry
Adaptor Proteins, Signal Transducing/metabolism ; Animals ; Humans ; Molecular Docking Simulation ; Protein Structure, Tertiary ; Rats ; Valosin Containing Protein/metabolism
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
A clinicopathologic study of malignancy in VCP-associated multisystem proteinopathy.
Autorzy:
Shmara A; Division of Genetic and Genomic Medicine, Department of Pediatrics, University of California-Irvine, Lab and FEDEX: Hewitt Hall, Rm 2038, Health Sciences Rd., Irvine, CA, 92697, USA.
Perez-Rosendahl M; Department of Pathology, University of California-Irvine, Irvine, CA, USA.
Murphy K; Division of Genetic and Genomic Medicine, Department of Pediatrics, University of California-Irvine, Lab and FEDEX: Hewitt Hall, Rm 2038, Health Sciences Rd., Irvine, CA, 92697, USA.
Kwon A; Division of Genetic and Genomic Medicine, Department of Pediatrics, University of California-Irvine, Lab and FEDEX: Hewitt Hall, Rm 2038, Health Sciences Rd., Irvine, CA, 92697, USA.
Smith C; Department of Neurology and Sanders-Brown Center On Aging, University of Kentucky, Lexington, KY, USA.
Kimonis V; Division of Genetic and Genomic Medicine, Department of Pediatrics, University of California-Irvine, Lab and FEDEX: Hewitt Hall, Rm 2038, Health Sciences Rd., Irvine, CA, 92697, USA. .
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Źródło:
Orphanet journal of rare diseases [Orphanet J Rare Dis] 2022 Jul 15; Vol. 17 (1), pp. 272. Date of Electronic Publication: 2022 Jul 15.
Typ publikacji:
Journal Article
MeSH Terms:
Myositis, Inclusion Body*/genetics
Myositis, Inclusion Body*/metabolism
Neoplasms*/genetics
Neoplasms*/metabolism
Valosin Containing Protein*/genetics
Valosin Containing Protein*/metabolism
Adenosine Triphosphatases/genetics ; Cell Cycle Proteins/genetics ; Cell Cycle Proteins/metabolism ; Humans ; Male ; Mutation ; Retrospective Studies
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
VCP inhibition induces an unfolded protein response and apoptosis in human acute myeloid leukemia cells.
Autorzy:
Szczęśniak PP; Department of Medicine, Hematology/Oncology, Goethe University, Frankfurt, Germany.
Heidelberger JB; Institute of Molecular Biology (IMB), Mainz, Germany.
Serve H; Department of Medicine, Hematology/Oncology, Goethe University, Frankfurt, Germany.; German Cancer Consortium (DKTK) and German Cancer Research Center (DKFZ), Heidelberg, Germany.; Frankfurt Cancer Institute (FCI), Frankfurt, Germany.
Beli P; Institute of Molecular Biology (IMB), Mainz, Germany.; Institute of Developmental Biology and Neurobiology (IDN), Johannes Gutenberg University Mainz, Mainz, Germany.
Wagner SA; Department of Medicine, Hematology/Oncology, Goethe University, Frankfurt, Germany.; German Cancer Consortium (DKTK) and German Cancer Research Center (DKFZ), Heidelberg, Germany.; Frankfurt Cancer Institute (FCI), Frankfurt, Germany.
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Źródło:
PloS one [PLoS One] 2022 Apr 06; Vol. 17 (4), pp. e0266478. Date of Electronic Publication: 2022 Apr 06 (Print Publication: 2022).
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Apoptosis*
Leukemia, Myeloid, Acute*/drug therapy
Leukemia, Myeloid, Acute*/genetics
Leukemia, Myeloid, Acute*/metabolism
Unfolded Protein Response*
Valosin Containing Protein*/metabolism
Cell Line, Tumor ; Humans ; Proteasome Endopeptidase Complex/metabolism
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Impacts of p97 on Proteome Changes in Human Cells during Coronaviral Replication.
Autorzy:
Cheng KW; Division of Biology and Biological Engineering, California Institute of Technology, Pasadena, CA 91125, USA.
Li S; Division of Biology and Biological Engineering, California Institute of Technology, Pasadena, CA 91125, USA.
Wang F; Division of Biology and Biological Engineering, California Institute of Technology, Pasadena, CA 91125, USA.
Ruiz-Lopez NM; Division of Biology and Biological Engineering, California Institute of Technology, Pasadena, CA 91125, USA.
Houerbi N; Division of Biology and Biological Engineering, California Institute of Technology, Pasadena, CA 91125, USA.
Chou TF; Division of Biology and Biological Engineering, California Institute of Technology, Pasadena, CA 91125, USA.; Proteome Exploration Laboratory, Beckman Institute, California Institute of Technology, Pasadena, CA 91125, USA.
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Źródło:
Cells [Cells] 2021 Oct 29; Vol. 10 (11). Date of Electronic Publication: 2021 Oct 29.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Coronavirus 229E, Human/*physiology
Coronavirus OC43, Human/*physiology
Valosin Containing Protein/*metabolism
Virus Replication/*physiology
Antiviral Agents/pharmacology ; Cell Cycle/drug effects ; Cell Line ; Coronavirus 229E, Human/drug effects ; Coronavirus OC43, Human/drug effects ; Cytopathogenic Effect, Viral/drug effects ; Humans ; Proteome/drug effects ; Proteome/metabolism ; RNA, Small Interfering/genetics ; RNA, Viral/biosynthesis ; Valosin Containing Protein/antagonists & inhibitors ; Valosin Containing Protein/genetics ; Virus Replication/drug effects ; Virus Uncoating/drug effects
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Functional Inhibition of Valosin-Containing Protein Induces Cardiac Dilation and Dysfunction in a New Dominant-Negative Transgenic Mouse Model.
Autorzy:
Sun X; Center for Molecular and Translational Medicine, Institute of Biomedical Science, Georgia State University, Atlanta, GA 30303, USA.
Zhou N; Division of Physiology, Department of Basic Sciences, School of Medicine, Loma Linda University, Loma Linda, CA 92318, USA.
Ma B; Center for Molecular and Translational Medicine, Institute of Biomedical Science, Georgia State University, Atlanta, GA 30303, USA.
Wu W; Center for Molecular and Translational Medicine, Institute of Biomedical Science, Georgia State University, Atlanta, GA 30303, USA.
Stoll S; Division of Physiology, Department of Basic Sciences, School of Medicine, Loma Linda University, Loma Linda, CA 92318, USA.
Lai L; Center for Molecular and Translational Medicine, Institute of Biomedical Science, Georgia State University, Atlanta, GA 30303, USA.
Qin G; Department of Biomedical Engineering, School of Medicine and School of Engineering, University of Alabama at Birmingham, Birmingham, AL 35294, USA.
Qiu H; Center for Molecular and Translational Medicine, Institute of Biomedical Science, Georgia State University, Atlanta, GA 30303, USA.; Division of Physiology, Department of Basic Sciences, School of Medicine, Loma Linda University, Loma Linda, CA 92318, USA.
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Źródło:
Cells [Cells] 2021 Oct 26; Vol. 10 (11). Date of Electronic Publication: 2021 Oct 26.
Typ publikacji:
Journal Article; Research Support, N.I.H., Extramural
MeSH Terms:
Cardiomyopathy, Dilated/*pathology
Cardiomyopathy, Dilated/*physiopathology
Valosin Containing Protein/*antagonists & inhibitors
Aging/pathology ; Animals ; Cell Nucleus/metabolism ; Disease Models, Animal ; Mice, Transgenic ; Myocardium/pathology ; Repressor Proteins/metabolism ; Valosin Containing Protein/metabolism
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
The Biogenesis of Dengue Virus Replication Organelles Requires the ATPase Activity of Valosin-Containing Protein.
Autorzy:
Mazeaud C; Centre Armand-Frappier Santé Biotechnologie, Institut National de la Recherche Scientifique, Laval, QC H7V 1B7, Canada.
Anton A; Centre Armand-Frappier Santé Biotechnologie, Institut National de la Recherche Scientifique, Laval, QC H7V 1B7, Canada.
Pahmeier F; Center for Integrative Infectious Disease Research (CIID), Department of Infectious Diseases, Molecular Virology, Heidelberg University, D-69120 Heidelberg, Germany.
Sow AA; Centre Armand-Frappier Santé Biotechnologie, Institut National de la Recherche Scientifique, Laval, QC H7V 1B7, Canada.
Cerikan B; Center for Integrative Infectious Disease Research (CIID), Department of Infectious Diseases, Molecular Virology, Heidelberg University, D-69120 Heidelberg, Germany.
Freppel W; Centre Armand-Frappier Santé Biotechnologie, Institut National de la Recherche Scientifique, Laval, QC H7V 1B7, Canada.
Cortese M; Center for Integrative Infectious Disease Research (CIID), Department of Infectious Diseases, Molecular Virology, Heidelberg University, D-69120 Heidelberg, Germany.
Bartenschlager R; Center for Integrative Infectious Disease Research (CIID), Department of Infectious Diseases, Molecular Virology, Heidelberg University, D-69120 Heidelberg, Germany.; German Center for Infection Research (DZIF), Heidelberg Partner Site, D-69120 Heidelberg, Germany.
Chatel-Chaix L; Centre Armand-Frappier Santé Biotechnologie, Institut National de la Recherche Scientifique, Laval, QC H7V 1B7, Canada.; Center of Excellence in Research on Orphan Diseases-Fondation Courtois, Montreal, QC H7V 1B7, Canada.; Réseau Intersectoriel de Recherche en Santé de l'Université du Québec, Québec, QC H7V 1B7, Canada.
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Źródło:
Viruses [Viruses] 2021 Oct 18; Vol. 13 (10). Date of Electronic Publication: 2021 Oct 18.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Dengue Virus/*metabolism
Valosin Containing Protein/*metabolism
Viral Replication Compartments/*physiology
Adenosine Triphosphatases/genetics ; Cell Line ; Dengue/virology ; Dengue Virus/genetics ; Dengue Virus/pathogenicity ; Endoplasmic Reticulum/virology ; Humans ; RNA, Viral/genetics ; Valosin Containing Protein/genetics ; Viral Nonstructural Proteins/genetics ; Virus Replication/physiology
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknieSzczegóły
Tytuł:
MEST promotes lung cancer invasion and metastasis by interacting with VCP to activate NF-κB signaling.
Autorzy:
Wang Y; MOE Key Laboratory of Tumor Molecular Biology and Key Laboratory of Functional Protein Research of Guangdong Higher Education Institutes, Institute of Life and Health Engineering, College of Life Science and Technology, Jinan University, Guangzhou, 510632, China.
Zhang J; MOE Key Laboratory of Tumor Molecular Biology and Key Laboratory of Functional Protein Research of Guangdong Higher Education Institutes, Institute of Life and Health Engineering, College of Life Science and Technology, Jinan University, Guangzhou, 510632, China.
Li YJ; MOE Key Laboratory of Tumor Molecular Biology and Key Laboratory of Functional Protein Research of Guangdong Higher Education Institutes, Institute of Life and Health Engineering, College of Life Science and Technology, Jinan University, Guangzhou, 510632, China.
Yu NN; MOE Key Laboratory of Tumor Molecular Biology and Key Laboratory of Functional Protein Research of Guangdong Higher Education Institutes, Institute of Life and Health Engineering, College of Life Science and Technology, Jinan University, Guangzhou, 510632, China.
Liu WT; MOE Key Laboratory of Tumor Molecular Biology and Key Laboratory of Functional Protein Research of Guangdong Higher Education Institutes, Institute of Life and Health Engineering, College of Life Science and Technology, Jinan University, Guangzhou, 510632, China.
Liang JZ; MOE Key Laboratory of Tumor Molecular Biology and Key Laboratory of Functional Protein Research of Guangdong Higher Education Institutes, Institute of Life and Health Engineering, College of Life Science and Technology, Jinan University, Guangzhou, 510632, China.
Xu WW; MOE Key Laboratory of Tumor Molecular Biology and Guangdong Provincial Key Laboratory of Bioengineering Medicine, National Engineering Research Center of Genetic Medicine, Institute of Biomedicine, Jinan University, Guangzhou, 510632, China.
Sun ZH; MOE Key Laboratory of Tumor Molecular Biology and Key Laboratory of Functional Protein Research of Guangdong Higher Education Institutes, Institute of Life and Health Engineering, College of Life Science and Technology, Jinan University, Guangzhou, 510632, China.
Li B; MOE Key Laboratory of Tumor Molecular Biology and Key Laboratory of Functional Protein Research of Guangdong Higher Education Institutes, Institute of Life and Health Engineering, College of Life Science and Technology, Jinan University, Guangzhou, 510632, China. .
He QY; MOE Key Laboratory of Tumor Molecular Biology and Key Laboratory of Functional Protein Research of Guangdong Higher Education Institutes, Institute of Life and Health Engineering, College of Life Science and Technology, Jinan University, Guangzhou, 510632, China. .
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Źródło:
Journal of experimental & clinical cancer research : CR [J Exp Clin Cancer Res] 2021 Sep 24; Vol. 40 (1), pp. 301. Date of Electronic Publication: 2021 Sep 24.
Typ publikacji:
Journal Article
MeSH Terms:
Gene Expression Regulation, Neoplastic*
Biomarkers, Tumor/*metabolism
Lung Neoplasms/*pathology
NF-kappa B/*metabolism
Proteins/*metabolism
Valosin Containing Protein/*metabolism
Animals ; Apoptosis ; Biomarkers, Tumor/genetics ; Cell Proliferation ; Female ; Humans ; Lung Neoplasms/genetics ; Lung Neoplasms/metabolism ; Male ; Mice ; Mice, Inbred BALB C ; Mice, Nude ; Middle Aged ; NF-kappa B/genetics ; Neoplasm Invasiveness ; Neoplasm Metastasis ; Prognosis ; Proteins/genetics ; Survival Rate ; Tumor Cells, Cultured ; Valosin Containing Protein/genetics ; Xenograft Model Antitumor Assays
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Valosin-Containing Protein (VCP)/p97: A Prognostic Biomarker and Therapeutic Target in Cancer.
Autorzy:
Costantini S; Experimental Pharmacology Unit-Laboratory of Mercogliano (AV), Istituto Nazionale Tumori-IRCCS Fondazione G. Pascale, 80131 Naples, Italy.
Capone F; Experimental Pharmacology Unit-Laboratory of Mercogliano (AV), Istituto Nazionale Tumori-IRCCS Fondazione G. Pascale, 80131 Naples, Italy.
Polo A; Experimental Pharmacology Unit-Laboratory of Mercogliano (AV), Istituto Nazionale Tumori-IRCCS Fondazione G. Pascale, 80131 Naples, Italy.
Bagnara P; Experimental Pharmacology Unit-Laboratory of Mercogliano (AV), Istituto Nazionale Tumori-IRCCS Fondazione G. Pascale, 80131 Naples, Italy.
Budillon A; Experimental Pharmacology Unit-Laboratory of Mercogliano (AV), Istituto Nazionale Tumori-IRCCS Fondazione G. Pascale, 80131 Naples, Italy.
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Źródło:
International journal of molecular sciences [Int J Mol Sci] 2021 Sep 21; Vol. 22 (18). Date of Electronic Publication: 2021 Sep 21.
Typ publikacji:
Journal Article; Review
MeSH Terms:
Cell Cycle Proteins/*metabolism
Valosin Containing Protein/*metabolism
Adenosine Triphosphatases/genetics ; Adenosine Triphosphatases/metabolism ; Animals ; Cell Cycle Proteins/genetics ; Humans ; Prognosis ; Protein Binding ; Valosin Containing Protein/genetics
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknieSzczegóły
Tytuł:
How Viruses Use the VCP/p97 ATPase Molecular Machine.
Autorzy:
Das P; Department of Molecular Biosciences, The University of Texas at Austin, Austin, TX 78712, USA.
Dudley JP; Department of Molecular Biosciences, The University of Texas at Austin, Austin, TX 78712, USA.; LaMontagne Center for Infectious Disease, The University of Texas at Austin, Austin, TX 78712, USA.
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Źródło:
Viruses [Viruses] 2021 Sep 21; Vol. 13 (9). Date of Electronic Publication: 2021 Sep 21.
Typ publikacji:
Journal Article; Research Support, N.I.H., Extramural; Review
MeSH Terms:
Virus Replication*
Adenosine Triphosphatases/*metabolism
Nuclear Proteins/*metabolism
Valosin Containing Protein/*metabolism
Virus Diseases/*virology
Viruses/*metabolism
Adenosine Triphosphatases/chemistry ; Endoplasmic Reticulum-Associated Degradation ; Genome, Viral/genetics ; Host-Pathogen Interactions ; Humans ; Nuclear Proteins/chemistry ; Valosin Containing Protein/chemistry ; Virus Diseases/immunology ; Virus Internalization
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Novel variants, muscle imaging, and myopathological changes in Chinese patients with VCP-related multisystem proteinopathy.
Autorzy:
Wan Y; Department of Neurology, Peking University First Hospital, 100034, Beijing, China.
Wang Q; Department of Neurology, Peking University First Hospital, 100034, Beijing, China.
Zheng Y; Department of Neurology, Peking University First Hospital, 100034, Beijing, China.
Yu M; Department of Neurology, Peking University First Hospital, 100034, Beijing, China.
Xie Z; Department of Neurology, Peking University First Hospital, 100034, Beijing, China.
Ling C; Department of Neurology, Peking University First Hospital, 100034, Beijing, China.
Meng L; Department of Neurology, Peking University First Hospital, 100034, Beijing, China.
Yu J; Department of Neurology, Peking University First Hospital, 100034, Beijing, China.
Zheng Y; Department of Neurology, the First Affiliated Hospital of Nanchang University, 330006, Jiangxi, Nanchang, 17 Yongwaizheng Street, China.
Wang Y; Department of Neurology, Peking University First Hospital, 100034, Beijing, China.
Zhang W; Department of Neurology, Peking University First Hospital, 100034, Beijing, China.
Liu C; Department of Neurology, Peking University First Hospital, 100034, Beijing, China.
Zhao Y; Department of Neurology, Peking University First Hospital, 100034, Beijing, China.
Yuan Y; Department of Neurology, Peking University First Hospital, 100034, Beijing, China.
Deng J; Department of Neurology, Peking University First Hospital, 100034, Beijing, China.
Gang Q; Department of Neurology, Peking University First Hospital, 100034, Beijing, China.; Department of Neurology, Peking University First Hospital, Peking University, 100034, Beijing, China.
Wang Z; Department of Neurology, Peking University First Hospital, 100034, Beijing, China.; Department of Neurology, Peking University First Hospital, Peking University, 100034, Beijing, China.
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Źródło:
Molecular genetics & genomic medicine [Mol Genet Genomic Med] 2023 Jul; Vol. 11 (7), pp. e2176. Date of Electronic Publication: 2023 Mar 31.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Cell Cycle Proteins*/genetics
Muscular Diseases*/genetics
Adult ; Humans ; Valosin Containing Protein/genetics ; East Asian People ; Muscle, Skeletal/diagnostic imaging ; Muscle, Skeletal/pathology
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
Provisional practice recommendation for the management of myopathy in VCP-associated multisystem proteinopathy.
Autorzy:
Roy B; Department of Neurology, Yale School of Medicine, New Haven, Connecticut, USA.
Peck A; Cure VCP Disease, Americus, Georgia, USA.
Evangelista T; GH Pitié-Salpêtrière, Sorbonne Université-Inserm UMRS97, Institut de Myologie, Paris, France.
Pfeffer G; Hotchkiss Brain Institute, Department of Clinical Neurosciences, University of Calgary Cumming School of Medicine, Calgary, Alberta, Canada.
Wang L; Department of Neurology, University of Washington, Seattle, Washington, USA.
Diaz-Manera J; John Walton Muscular Dystrophy Research Centre, Newcastle University, Newcastle upon Tyne, UK.
Korb M; Department of Neurology, University of California-Irvine School of Medicine, Orange, California, USA.
Wicklund MP; Department of Neurology, University of Colorado, Denver, Colorado, USA.
Milone M; Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
Freimer M; Department of Neurology, Ohio State University, Columbus, Ohio, USA.
Kushlaf H; Department of Neurology and Rehabilitation Medicine, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA.
Villar-Quiles RN; APHP, Reference Center for Neuromuscular Disorders, Center of Research in Myology, Sorbonne Université-Inserm UMRS974, Pitié-Salpêtrière Hospital, Paris, France.
Stojkovic T; APHP, Reference Center for Neuromuscular Disorders, Center of Research in Myology, Sorbonne Université-Inserm UMRS974, Pitié-Salpêtrière Hospital, Paris, France.
Needham M; University of Notre Dame, Murdoch University and Fiona Stanley Hospital, Perth, Australia.
Palmio J; Neuromuscular Research Center, Tampere University Hospital, Tampere, Finland.
Lloyd TE; Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Massachusetts, USA.; Department of Neuroscience and Pathology, Johns Hopkins University School of Medicine, Baltimore, Massachusetts, USA.
Keung B; Department of Neurology, Yale School of Medicine, New Haven, Connecticut, USA.
Mozaffar T; Department of Neurology, University of California-Irvine School of Medicine, Orange, California, USA.
Weihl CC; Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA.
Kimonis V; Department of Neurology, University of California-Irvine School of Medicine, Orange, California, USA.; Department of Pediatrics, University of California-Irvine School of Medicine, Orange, California, USA.
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Źródło:
Annals of clinical and translational neurology [Ann Clin Transl Neurol] 2023 May; Vol. 10 (5), pp. 686-695. Date of Electronic Publication: 2023 Apr 07.
Typ publikacji:
Journal Article; Review
MeSH Terms:
Muscular Diseases*/diagnosis
Muscular Diseases*/genetics
Muscular Diseases*/therapy
Muscular Dystrophies, Limb-Girdle*/diagnosis
Muscular Dystrophies, Limb-Girdle*/genetics
Muscular Dystrophies, Limb-Girdle*/therapy
Proteostasis Deficiencies*
Humans ; Valosin Containing Protein/genetics ; Phenotype
Czasopismo naukowe
Pełny tekst  Link otwiera się w nowym oknie Pełny tekstSzczegóły
Tytuł:
An autopsy report of a familial amyotrophic lateral sclerosis case carrying VCP Arg487His mutation with a unique TDP-43 proteinopathy.
Autorzy:
Matsubara T; Department of Neurology, Mifukai Vihara Hananosato Hospital, Hiroshima, Japan.; Department of Neurology and Neuropathology (The Brain Bank for Aging Research), Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology, Tokyo, Japan.; Department of Clinical Neuroscience and Therapeutics, Hiroshima University Graduate School of Biomedical and Health Sciences, Hiroshima, Japan.
Izumi Y; Department of Neurology, Mifukai Vihara Hananosato Hospital, Hiroshima, Japan.; Department of Neurology, Tokushima University Graduate School of Biomedical Sciences, Tokushima, Japan.
Oda M; Department of Neurology, Mifukai Vihara Hananosato Hospital, Hiroshima, Japan.
Takahashi M; Department of Neurology, Shinko Hospital, Kobe, Japan.
Maruyama H; Department of Clinical Neuroscience and Therapeutics, Hiroshima University Graduate School of Biomedical and Health Sciences, Hiroshima, Japan.
Miyamoto R; Department of Neurology, Tokushima University Graduate School of Biomedical Sciences, Tokushima, Japan.
Watanabe C; Department of Neurology, National Hospital Organization Hiroshima-Nishi Medical Center, Hiroshima, Japan.
Tachiyama Y; Department of Clinical Laboratory, National Hospital Organization Hiroshima-Nishi Medical Center, Hiroshima, Japan.
Morino H; Department of Clinical Neuroscience and Therapeutics, Hiroshima University Graduate School of Biomedical and Health Sciences, Hiroshima, Japan.; Department of Epidemiology, Research Institute for Radiation Biology and Medicine, Hiroshima University, Hiroshima, Japan.
Kawakami H; Department of Epidemiology, Research Institute for Radiation Biology and Medicine, Hiroshima University, Hiroshima, Japan.
Saito Y; Department of Neurology and Neuropathology (The Brain Bank for Aging Research), Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology, Tokyo, Japan.
Murayama S; Department of Neurology and Neuropathology (The Brain Bank for Aging Research), Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology, Tokyo, Japan.; Molecular Research Center for Children's Mental Development (Brain Bank for Neurodevelopmental, Neurological and Psychiatric Disorders), United Graduate School of Child Development, Osaka University, Osaka, Japan.
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Źródło:
Neuropathology : official journal of the Japanese Society of Neuropathology [Neuropathology] 2021 Apr; Vol. 41 (2), pp. 118-126. Date of Electronic Publication: 2021 Jan 07.
Typ publikacji:
Case Reports
MeSH Terms:
Autopsy*/methods
Amyotrophic Lateral Sclerosis/*metabolism
Amyotrophic Lateral Sclerosis/*pathology
Mutation/*genetics
TDP-43 Proteinopathies/*metabolism
Valosin Containing Protein/*genetics
DNA-Binding Proteins/metabolism ; Frontotemporal Lobar Degeneration/metabolism ; Frontotemporal Lobar Degeneration/pathology ; Humans ; Intranuclear Inclusion Bodies/metabolism ; Male ; Middle Aged ; Motor Neurons/pathology ; Valosin Containing Protein/metabolism
Raport
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Tytuł:
Valosin-containing protein ATPase activity regulates the morphogenesis of Zika virus replication organelles and virus-induced cell death.
Autorzy:
Anton A; Centre Armand-Frappier Santé Biotechnologie, Institut National de la Recherche Scientifique, Laval, Québec, Canada.
Mazeaud C; Centre Armand-Frappier Santé Biotechnologie, Institut National de la Recherche Scientifique, Laval, Québec, Canada.
Freppel W; Centre Armand-Frappier Santé Biotechnologie, Institut National de la Recherche Scientifique, Laval, Québec, Canada.
Gilbert C; Centre Armand-Frappier Santé Biotechnologie, Institut National de la Recherche Scientifique, Laval, Québec, Canada.
Tremblay N; Centre Armand-Frappier Santé Biotechnologie, Institut National de la Recherche Scientifique, Laval, Québec, Canada.
Sow AA; Centre Armand-Frappier Santé Biotechnologie, Institut National de la Recherche Scientifique, Laval, Québec, Canada.
Roy M; Centre Armand-Frappier Santé Biotechnologie, Institut National de la Recherche Scientifique, Laval, Québec, Canada.
Rodrigue-Gervais IG; Centre Armand-Frappier Santé Biotechnologie, Institut National de la Recherche Scientifique, Laval, Québec, Canada.
Chatel-Chaix L; Centre Armand-Frappier Santé Biotechnologie, Institut National de la Recherche Scientifique, Laval, Québec, Canada.; Center of Excellence in Research on Orphan Diseases-Courtois Foundation (CERMO-FC), Montreal, Québec, Canada.; Réseau Intersectoriel de Recherche en Santé de l'Université du Québec (RISUQ), Québec, Canada.
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Źródło:
Cellular microbiology [Cell Microbiol] 2021 Apr; Vol. 23 (4), pp. e13302. Date of Electronic Publication: 2021 Jan 11.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Apoptosis*
Gene Expression Regulation*
Adenosine Triphosphatases/*metabolism
Valosin Containing Protein/*genetics
Valosin Containing Protein/*metabolism
Zika Virus/*genetics
Zika Virus/*physiology
Acetanilides/pharmacology ; Adenosine Triphosphatases/genetics ; Animals ; Benzothiazoles/pharmacology ; Cell Line, Tumor ; Chlorocebus aethiops ; HEK293 Cells ; Host Microbial Interactions/drug effects ; Host Microbial Interactions/genetics ; Humans ; Indoles/pharmacology ; Mitochondria/drug effects ; Mitochondria/metabolism ; Mitochondria/virology ; Pyrimidines/pharmacology ; Valosin Containing Protein/antagonists & inhibitors ; Vero Cells
Czasopismo naukowe
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Tytuł:
Structural and Functional Analysis of Disease-Linked p97 ATPase Mutant Complexes.
Autorzy:
Nandi P; Biodesign Center for Applied Structural Discovery, School of Molecular Sciences, Arizona State University, Tempe, AZ 85287, USA.
Li S; Division of Biology and Biological Engineering, California Institute of Technology, Pasadena, CA 91125, USA.
Columbres RCA; Division of Biology and Biological Engineering, California Institute of Technology, Pasadena, CA 91125, USA.
Wang F; Division of Biology and Biological Engineering, California Institute of Technology, Pasadena, CA 91125, USA.
Williams DR; Eyring Materials Center, Arizona State University, Tempe, AZ 85287, USA.
Poh YP; Division of Biology and Biological Engineering, California Institute of Technology, Pasadena, CA 91125, USA.
Chou TF; Division of Biology and Biological Engineering, California Institute of Technology, Pasadena, CA 91125, USA.
Chiu PL; Biodesign Center for Applied Structural Discovery, School of Molecular Sciences, Arizona State University, Tempe, AZ 85287, USA.
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Źródło:
International journal of molecular sciences [Int J Mol Sci] 2021 Jul 28; Vol. 22 (15). Date of Electronic Publication: 2021 Jul 28.
Typ publikacji:
Journal Article
MeSH Terms:
Models, Molecular*
Mutation*
Frontotemporal Dementia/*metabolism
Muscular Dystrophies, Limb-Girdle/*metabolism
Myositis, Inclusion Body/*metabolism
Osteitis Deformans/*metabolism
Soluble N-Ethylmaleimide-Sensitive Factor Attachment Proteins/*metabolism
Valosin Containing Protein/*genetics
Frontotemporal Dementia/genetics ; Humans ; Microscopy, Electron, Transmission ; Muscular Dystrophies, Limb-Girdle/genetics ; Myositis, Inclusion Body/genetics ; Osteitis Deformans/genetics ; Protein Conformation ; Valosin Containing Protein/metabolism
SCR Disease Name:
Inclusion Body Myopathy With Early-Onset Paget Disease And Frontotemporal Dementia
Czasopismo naukowe
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Tytuł:
Valosin-containing protein Asp395Gly mutation in a patient with frontotemporal dementia: a case report.
Autorzy:
Kobayashi R; Department of Psychiatry, Yamagata University School of Medicine, 2-2-2 Iidanishi, Yamagata, 990-9585, Japan. .
Naruse H; Department of Neurology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.
Kawakatsu S; Department of Neuropsychiatry, Aizu Medical Center, Fukushima Medical University, Aizuwakamatsu, Japan.
Iseki C; Department of Neurology, Hematology, Metabolism, Endocrinology, and Diabetology, Yamagata University School of Medicine, Yamagata, Japan.
Suzuki Y; Department of Neurology, Hematology, Metabolism, Endocrinology, and Diabetology, Yamagata University School of Medicine, Yamagata, Japan.
Koyama S; Department of Neurology, Hematology, Metabolism, Endocrinology, and Diabetology, Yamagata University School of Medicine, Yamagata, Japan.
Morioka D; Department of Psychiatry, Yamagata University School of Medicine, 2-2-2 Iidanishi, Yamagata, 990-9585, Japan.
Ishiura H; Department of Neurology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.
Mitsui J; Department of Neurology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.; Department of Molecular Neurology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.
Ohta Y; Department of Neurology, Hematology, Metabolism, Endocrinology, and Diabetology, Yamagata University School of Medicine, Yamagata, Japan.
Tsuji S; Department of Molecular Neurology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.; Institute of Medical Genomics, International University of Health and Welfare, Chiba, Japan.
Toda T; Department of Neurology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.
Otani K; Department of Psychiatry, Yamagata University School of Medicine, 2-2-2 Iidanishi, Yamagata, 990-9585, Japan.
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Źródło:
BMC neurology [BMC Neurol] 2022 Nov 03; Vol. 22 (1), pp. 406. Date of Electronic Publication: 2022 Nov 03.
Typ publikacji:
Case Reports; Journal Article
MeSH Terms:
Frontotemporal Dementia*/complications
Frontotemporal Dementia*/genetics
Frontotemporal Dementia*/pathology
Frontotemporal Lobar Degeneration*
Bone Diseases*
Male ; Humans ; Adult ; Middle Aged ; Valosin Containing Protein/genetics ; Valosin Containing Protein/metabolism ; Mutation/genetics ; DNA-Binding Proteins/genetics
Czasopismo naukowe
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Tytuł:
Multisystem proteinopathies (MSPs) and MSP-like disorders: Clinical-pathological-molecular spectrum.
Autorzy:
Chompoopong P; Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
Oskarsson B; Department of Neurology, Mayo Clinic, Jacksonville, Florida, USA.
Madigan NN; Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
Mirman I; Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
Martinez-Thompson JM; Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
Liewluck T; Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
Milone M; Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
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Źródło:
Annals of clinical and translational neurology [Ann Clin Transl Neurol] 2023 Apr; Vol. 10 (4), pp. 632-643. Date of Electronic Publication: 2023 Mar 01.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Frontotemporal Dementia*/genetics
Muscular Diseases*/genetics
Humans ; Middle Aged ; Valosin Containing Protein/genetics ; Sequestosome-1 Protein/genetics ; RNA-Binding Proteins ; Nuclear Matrix-Associated Proteins
SCR Disease Name:
Vacuolar myopathy
Czasopismo naukowe
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Tytuł:
Autosomal dominant VCP hypomorph mutation impairs disaggregation of PHF-tau.
Autorzy:
Darwich NF; Translational Neuropathology Research Laboratory, Department of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, PA, USA.
Phan JM; Translational Neuropathology Research Laboratory, Department of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, PA, USA.
Kim B; Translational Neuropathology Research Laboratory, Department of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, PA, USA.
Suh E; Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, PA, USA.
Papatriantafyllou JD; Medical Center of Athens, Memory Disorders Clinic and Day Care Center for Third Age 'IASIS,' Athens, Greece.
Changolkar L; Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, PA, USA.
Nguyen AT; Translational Neuropathology Research Laboratory, Department of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, PA, USA.
O'Rourke CM; Translational Neuropathology Research Laboratory, Department of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, PA, USA.
He Z; Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, PA, USA.
Porta S; Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, PA, USA.
Gibbons GS; Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, PA, USA.
Luk KC; Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, PA, USA.
Papageorgiou SG; First University Department of Neurology, Eginiteio University Hospital, National and Kapodistrian University of Athens, Athens, Greece.
Grossman M; Department of Neurology, Perelman School of Medicine at the University of Pennsylvania, PA, USA.
Massimo L; Department of Neurology, Perelman School of Medicine at the University of Pennsylvania, PA, USA.
Irwin DJ; Department of Neurology, Perelman School of Medicine at the University of Pennsylvania, PA, USA.
McMillan CT; Department of Neurology, Perelman School of Medicine at the University of Pennsylvania, PA, USA.
Nasrallah IM; Department of Radiology, Perelman School of Medicine at the University of Pennsylvania, PA, USA.
Toro C; NIH Undiagnosed Diseases Program, National Human Genome Research Institute, MD, USA.
Aguirre GK; Department of Neurology, Perelman School of Medicine at the University of Pennsylvania, PA, USA.
Van Deerlin VM; Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, PA, USA.
Lee EB; Translational Neuropathology Research Laboratory, Department of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, PA, USA. .
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Źródło:
Science (New York, N.Y.) [Science] 2020 Nov 20; Vol. 370 (6519). Date of Electronic Publication: 2020 Oct 01.
Typ publikacji:
Journal Article; Research Support, N.I.H., Extramural
MeSH Terms:
Protein Aggregates*
Alzheimer Disease/*metabolism
Alzheimer Disease/*pathology
Protein Aggregation, Pathological/*genetics
Valosin Containing Protein/*metabolism
tau Proteins/*metabolism
Animals ; Aspartic Acid/genetics ; Gene Knock-In Techniques ; Genes, Dominant ; Glycine/genetics ; Humans ; Mice, Inbred C57BL ; Mice, Mutant Strains ; Mutation ; Neurofibrillary Tangles/genetics ; Neurofibrillary Tangles/metabolism ; Phosphorylation ; Valosin Containing Protein/genetics
Czasopismo naukowe
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Tytuł:
Mitochondrial Surveillance by Cdc48/p97: MAD vs. Membrane Fusion.
Autorzy:
Escobar-Henriques M; Institute for Genetics, Cologne Excellence Cluster on Cellular Stress Responses in Aging-Associated Diseases (CECAD), Center for Molecular Medicine Cologne (CMMC), University of Cologne, Joseph-Stelzmann-Straße 26, 50931 Cologne, Germany.
Anton V; Institute for Genetics, Cologne Excellence Cluster on Cellular Stress Responses in Aging-Associated Diseases (CECAD), Center for Molecular Medicine Cologne (CMMC), University of Cologne, Joseph-Stelzmann-Straße 26, 50931 Cologne, Germany.
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Źródło:
International journal of molecular sciences [Int J Mol Sci] 2020 Sep 18; Vol. 21 (18). Date of Electronic Publication: 2020 Sep 18.
Typ publikacji:
Journal Article; Review
MeSH Terms:
Adenosine Triphosphatases/*metabolism
Endoplasmic Reticulum/*metabolism
Mitochondria/*metabolism
Mitochondrial Membranes/*metabolism
Nuclear Proteins/*metabolism
Valosin Containing Protein/*metabolism
Adenosine Triphosphatases/genetics ; Endoplasmic Reticulum-Associated Degradation ; GTP Phosphohydrolases/metabolism ; Membrane Fusion ; Mitochondrial Membrane Transport Proteins/metabolism ; Nuclear Proteins/genetics ; Proteasome Endopeptidase Complex/metabolism ; Ubiquitin/metabolism ; Valosin Containing Protein/genetics
Czasopismo naukowe
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Wyświetlanie 1-20 z 819

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