دورية أكاديمية
أعرض في EDS

A protein quality control pathway regulated by linear ubiquitination.

التفاصيل البيبلوغرافية
العنوان: A protein quality control pathway regulated by linear ubiquitination.
المؤلفون: van Well EM; Department of Molecular Cell Biology, Institute of Biochemistry and Pathobiochemistry, Ruhr University Bochum, Bochum, Germany., Bader V; Department of Molecular Cell Biology, Institute of Biochemistry and Pathobiochemistry, Ruhr University Bochum, Bochum, Germany., Patra M; Neurobiochemistry, Adolf Butenandt Institute, Ludwig-Maximilians-University Munich, Munich, Germany., Sánchez-Vicente A; Department of Molecular Cell Biology, Institute of Biochemistry and Pathobiochemistry, Ruhr University Bochum, Bochum, Germany., Meschede J; Department of Molecular Cell Biology, Institute of Biochemistry and Pathobiochemistry, Ruhr University Bochum, Bochum, Germany., Furthmann N; Department of Molecular Cell Biology, Institute of Biochemistry and Pathobiochemistry, Ruhr University Bochum, Bochum, Germany., Schnack C; Department of Molecular Cell Biology, Institute of Biochemistry and Pathobiochemistry, Ruhr University Bochum, Bochum, Germany., Blusch A; Department of Neurology, St Josef Hospital, Ruhr University Bochum, Bochum, Germany., Longworth J; Proteome and Genome Research Unit, Department of Oncology, Luxembourg Institute of Health, Strassen, Luxembourg., Petrasch-Parwez E; Department of Neuroanatomy and Molecular Brain Research, Ruhr University Bochum, Bochum, Germany., Mori K; Biomedical Center (BMC), Ludwig-Maximilians-University Munich, Munich, Germany., Arzberger T; Department of Psychiatry and Psychotherapy, Ludwig-Maximilians-University Munich, Munich, Germany.; Centre for Neuropathology and Prion Research, Ludwig-Maximilians-University Munich, Munich, Germany.; German Center for Neurodegenerative Diseases (DZNE) Munich, Munich, Germany., Trümbach D; Institute of Developmental Genetics, Helmholtz Zentrum München, German Research Center for Environmental Health, Neuherberg, Germany., Angersbach L; Department of Molecular Cell Biology, Institute of Biochemistry and Pathobiochemistry, Ruhr University Bochum, Bochum, Germany., Showkat C; Department of Molecular Cell Biology, Institute of Biochemistry and Pathobiochemistry, Ruhr University Bochum, Bochum, Germany., Sehr DA; Department of Molecular Cell Biology, Institute of Biochemistry and Pathobiochemistry, Ruhr University Bochum, Bochum, Germany., Berlemann LA; Department of Molecular Cell Biology, Institute of Biochemistry and Pathobiochemistry, Ruhr University Bochum, Bochum, Germany., Goldmann P; Department of Molecular Cell Biology, Institute of Biochemistry and Pathobiochemistry, Ruhr University Bochum, Bochum, Germany., Clement AM; Institute for Pathobiochemistry, University Medical Center of the Johannes Gutenberg-University, Mainz, Germany., Behl C; Institute for Pathobiochemistry, University Medical Center of the Johannes Gutenberg-University, Mainz, Germany., Woerner AC; Department of Cellular Biochemistry, Max Planck Institute of Biochemistry, Martinsried, Germany., Saft C; Department of Neurology, St Josef Hospital, Ruhr University Bochum, Bochum, Germany., Wurst W; German Center for Neurodegenerative Diseases (DZNE) Munich, Munich, Germany.; Institute of Developmental Genetics, Helmholtz Zentrum München, German Research Center for Environmental Health, Neuherberg, Germany.; Developmental Genetics, Technical University Munich, Neuherberg, Germany.; Munich Cluster for Systems Neurology (SyNergy), Munich, Germany., Haass C; Biomedical Center (BMC), Ludwig-Maximilians-University Munich, Munich, Germany.; German Center for Neurodegenerative Diseases (DZNE) Munich, Munich, Germany.; Munich Cluster for Systems Neurology (SyNergy), Munich, Germany., Ellrichmann G; Department of Neurology, St Josef Hospital, Ruhr University Bochum, Bochum, Germany., Gold R; Department of Neurology, St Josef Hospital, Ruhr University Bochum, Bochum, Germany., Dittmar G; Proteome and Genome Research Unit, Department of Oncology, Luxembourg Institute of Health, Strassen, Luxembourg., Hipp MS; Department of Cellular Biochemistry, Max Planck Institute of Biochemistry, Martinsried, Germany.; Munich Cluster for Systems Neurology (SyNergy), Munich, Germany., Hartl FU; Department of Cellular Biochemistry, Max Planck Institute of Biochemistry, Martinsried, Germany.; Munich Cluster for Systems Neurology (SyNergy), Munich, Germany., Tatzelt J; Neurobiochemistry, Adolf Butenandt Institute, Ludwig-Maximilians-University Munich, Munich, Germany.; Department of Biochemistry of Neurodegenerative Diseases, Institute of Biochemistry and Pathobiochemistry, Ruhr University Bochum, Bochum, Germany.; Cluster of Excellence RESOLV, Bochum, Germany., Winklhofer KF; Department of Molecular Cell Biology, Institute of Biochemistry and Pathobiochemistry, Ruhr University Bochum, Bochum, Germany konstanze.winklhofer@rub.de.; Neurobiochemistry, Adolf Butenandt Institute, Ludwig-Maximilians-University Munich, Munich, Germany.; German Center for Neurodegenerative Diseases (DZNE) Munich, Munich, Germany.; Munich Cluster for Systems Neurology (SyNergy), Munich, Germany.; Cluster of Excellence RESOLV, Bochum, Germany.
المصدر: The EMBO journal [EMBO J] 2019 May 02; Vol. 38 (9). Date of Electronic Publication: 2019 Mar 18.
نوع المنشور: Journal Article; Research Support, Non-U.S. Gov't
اللغة: English
بيانات الدورية: Publisher: Nature Publishing Group Country of Publication: England NLM ID: 8208664 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1460-2075 (Electronic) Linking ISSN: 02614189 NLM ISO Abbreviation: EMBO J Subsets: MEDLINE
أسماء مطبوعة: Publication: 2024- : [London] : Nature Publishing Group
Original Publication: Eynsham, Oxford, England : Published for the European Molecular Biology Organization by IRL Press, [c1982-
مواضيع طبية MeSH: Protein Processing, Post-Translational*, Huntingtin Protein/*metabolism , Huntington Disease/*metabolism , Polyubiquitin/*metabolism , Sp1 Transcription Factor/*metabolism , Valosin Containing Protein/*metabolism, Adult ; Aged ; Animals ; Brain/metabolism ; Brain/pathology ; Case-Control Studies ; Cells, Cultured ; Embryo, Mammalian/cytology ; Embryo, Mammalian/metabolism ; Female ; Fibroblasts/cytology ; Fibroblasts/metabolism ; Humans ; Huntingtin Protein/genetics ; Huntington Disease/genetics ; Huntington Disease/pathology ; Male ; Mice ; Mice, Knockout ; Middle Aged ; NF-kappa B/genetics ; NF-kappa B/metabolism ; Neurons/metabolism ; Neurons/pathology ; Protein Binding ; Protein Interaction Domains and Motifs ; Signal Transduction ; Sp1 Transcription Factor/genetics ; Ubiquitination ; Valosin Containing Protein/genetics
مستخلص: Neurodegenerative diseases are characterized by the accumulation of misfolded proteins in the brain. Insights into protein quality control mechanisms to prevent neuronal dysfunction and cell death are crucial in developing causal therapies. Here, we report that various disease-associated protein aggregates are modified by the linear ubiquitin chain assembly complex (LUBAC). HOIP, the catalytic component of LUBAC, is recruited to misfolded Huntingtin in a p97/VCP-dependent manner, resulting in the assembly of linear polyubiquitin. As a consequence, the interactive surface of misfolded Huntingtin species is shielded from unwanted interactions, for example with the low complexity sequence domain-containing transcription factor Sp1, and proteasomal degradation of misfolded Huntingtin is facilitated. Notably, all three core LUBAC components are transcriptionally regulated by Sp1, linking defective LUBAC expression to Huntington's disease. In support of a protective activity of linear ubiquitination, silencing of OTULIN, a deubiquitinase with unique specificity for linear polyubiquitin, decreases proteotoxicity, whereas silencing of HOIP has the opposite effect. These findings identify linear ubiquitination as a protein quality control mechanism and hence a novel target for disease-modifying strategies in proteinopathies.
(© 2019 The Authors.)
التعليقات: Comment in: EMBO J. 2019 May 2;38(9):. (PMID: 30975687)
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فهرسة مساهمة: Keywords: LUBAC; OTULIN; Huntingtin; p97; protein aggregation
المشرفين على المادة: 0 (HTT protein, human)
0 (Huntingtin Protein)
0 (NF-kappa B)
0 (Sp1 Transcription Factor)
0 (SP1 protein, human)
120904-94-1 (Polyubiquitin)
EC 3.6.4.6 (Valosin Containing Protein)
تواريخ الأحداث: Date Created: 20190320 Date Completed: 20200106 Latest Revision: 20231213
رمز التحديث: 20231215
مُعرف محوري في PubMed: PMC6484417
DOI: 10.15252/embj.2018100730
PMID: 30886048
قاعدة البيانات: MEDLINE
الوصف
تدمد:1460-2075
DOI:10.15252/embj.2018100730