ALS/FTD mutations in UBQLN2 impede autophagy by reducing autophagosome acidification through loss of function
出版年份 2020 全文链接
标题
ALS/FTD mutations in UBQLN2 impede autophagy by reducing autophagosome acidification through loss of function
作者
关键词
-
出版物
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
Volume 117, Issue 26, Pages 15230-15241
出版商
Proceedings of the National Academy of Sciences
发表日期
2020-06-09
DOI
10.1073/pnas.1917371117
参考文献
相关参考文献
注意:仅列出部分参考文献,下载原文获取全部文献信息。- SQSTM1/p62: a potential target for neurodegenerative disease
- (2019) Shifan Ma et al. ACS Chemical Neuroscience
- Structure of hRpn10 Bound to UBQLN2 UBL Illustrates Basis for Complementarity between Shuttle Factors and Substrates at the Proteasome
- (2019) Xiang Chen et al. JOURNAL OF MOLECULAR BIOLOGY
- Ubiquilins regulate autophagic flux through mTOR signalling and lysosomal acidification
- (2019) Mümine Şentürk et al. NATURE CELL BIOLOGY
- Lysosomal dysfunction in Down syndrome is APP-dependent and mediated by APP-βCTF (C99)
- (2019) Jiang Ying et al. JOURNAL OF NEUROSCIENCE
- ALS-Linked Mutations Affect UBQLN2 Oligomerization and Phase Separation in a Position- and Amino Acid-Dependent Manner
- (2019) Thuy P. Dao et al. STRUCTURE
- Overriding FUS autoregulation in mice triggers gain-of-toxic dysfunctions in RNA metabolism and autophagy-lysosome axis
- (2019) Shuo-Chien Ling et al. eLife
- Lou Gehrig’s Disease (ALS): UBQLN2 Mutations Strike Out of Phase
- (2019) Nicole Higgins et al. STRUCTURE
- Overexpression of human Atp13a2Isoform-1 protein protects cells against manganese and starvation-induced toxicity
- (2019) Janet Ugolino et al. PLoS One
- The specificity of ubiquitin binding to ubiquilin-1 is regulated by sequences besides its UBA domain
- (2019) Christine A. Harman et al. BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS
- Ubiquitin Modulates Liquid-Liquid Phase Separation of UBQLN2 via Disruption of Multivalent Interactions
- (2018) Thuy P. Dao et al. MOLECULAR CELL
- The MTM1–UBQLN2–HSP complex mediates degradation of misfolded intermediate filaments in skeletal muscle
- (2018) Christos Gavriilidis et al. NATURE CELL BIOLOGY
- Mechanism and medical implications of mammalian autophagy
- (2018) Ivan Dikic et al. NATURE REVIEWS MOLECULAR CELL BIOLOGY
- Lysosome trafficking and signaling in health and neurodegenerative diseases
- (2018) Pearl P.Y. Lie et al. NEUROBIOLOGY OF DISEASE
- ALS Genes in the Genomic Era and their Implications for FTD
- (2018) Hung Phuoc Nguyen et al. TRENDS IN GENETICS
- Striking phenotypic variation in a family with the P506S UBQLN2 mutation including amyotrophic lateral sclerosis, spastic paraplegia and frontotemporal dementia
- (2018) Soragia Athina Gkazi et al. NEUROBIOLOGY OF AGING
- Mutant UBQLN2 promotes toxicity by modulating intrinsic self-assembly
- (2018) Lisa M. Sharkey et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- Ubiquilin 2 modulates ALS/FTD-linked FUS–RNA complex dynamics and stress granule formation
- (2018) Elizabeth J. Alexander et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- Monitoring and Measuring Autophagy
- (2017) Saori R. Yoshii et al. INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
- The STI and UBA Domains of UBQLN1 Are Critical Determinants of Substrate Interaction and Proteostasis
- (2017) Zimple Kurlawala et al. JOURNAL OF CELLULAR BIOCHEMISTRY
- C9ORF72 and UBQLN2 mutations are causes of amyotrophic lateral sclerosis in New Zealand: a genetic and pathologic study using banked human brain tissue
- (2017) Emma L. Scotter et al. NEUROBIOLOGY OF AGING
- Distinct roles for motor neuron autophagy early and late in the SOD1G93Amouse model of ALS
- (2017) Noam D. Rudnick et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- Ubiquilin1 promotes antigen-receptor mediated proliferation by eliminating mislocalized mitochondrial proteins
- (2017) Alexandra M Whiteley et al. eLife
- A novel ALS-associated variant in UBQLN4 regulates motor axon morphogenesis
- (2017) Brittany M Edens et al. eLife
- Protein Quality Control and the Amyotrophic Lateral Sclerosis/Frontotemporal Dementia Continuum
- (2017) Hamideh Shahheydari et al. Frontiers in Molecular Neuroscience
- Autophagy Dysregulation in ALS: When Protein Aggregates Get Out of Hand
- (2017) Nandini Ramesh et al. Frontiers in Molecular Neuroscience
- UBQLN2 Mediates Autophagy-Independent Protein Aggregate Clearance by the Proteasome
- (2016) Roland Hjerpe et al. CELL
- Ubiquilins Chaperone and Triage Mitochondrial Membrane Proteins for Degradation
- (2016) Eisuke Itakura et al. MOLECULAR CELL
- Motor neuron disease, TDP-43 pathology, and memory deficits in mice expressing ALS–FTD-linkedUBQLN2mutations
- (2016) Nhat T. T. Le et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- The Biology of Proteostasis in Aging and Disease
- (2015) Johnathan Labbadia et al. Annual Review of Biochemistry
- Signature changes in ubiquilin expression in the R6/2 mouse model of Huntington’s disease
- (2015) Nathaniel Safren et al. BRAIN RESEARCH
- ALS-linked mutations in ubiquilin-2 or hnRNPA1 reduce interaction between ubiquilin-2 and hnRNPA1
- (2015) Kathleen M. Gilpin et al. HUMAN MOLECULAR GENETICS
- Defective Proteasome Delivery of Polyubiquitinated Proteins by Ubiquilin-2 Proteins Containing ALS Mutations
- (2015) Lydia Chang et al. PLoS One
- Pathogenic Ubqln2 gains toxic properties to induce neuron death
- (2014) Qinxue Wu et al. ACTA NEUROPATHOLOGICA
- Common pathobiochemical hallmarks of progranulin-associated frontotemporal lobar degeneration and neuronal ceroid lipofuscinosis
- (2014) Julia K. Götzl et al. ACTA NEUROPATHOLOGICA
- Eukaryotic V-ATPase: Novel structural findings and functional insights
- (2014) Vladimir Marshansky et al. BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS
- RILP regulates vacuolar ATPase through interaction with the V1G1 subunit
- (2014) M. De Luca et al. JOURNAL OF CELL SCIENCE
- The vacuolar-type H+-ATPase at a glance - more than a proton pump
- (2014) M. E. Maxson et al. JOURNAL OF CELL SCIENCE
- Dendritic spinopathy in transgenic mice expressing ALS/dementia-linked mutantUBQLN2
- (2014) George H. Gorrie et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- Protein recycling pathways in neurodegenerative diseases
- (2014) Faisal Fecto et al. Alzheimers Research & Therapy
- Ubiquilin4 is an adaptor protein that recruits Ubiquilin1 to the autophagy machinery
- (2013) Dong Yun Lee et al. EMBO REPORTS
- Pathogenic mutation of UBQLN2 impairs its interaction with UBXD8 and disrupts endoplasmic reticulum-associated protein degradation
- (2013) Yuxing Xia et al. JOURNAL OF NEUROCHEMISTRY
- Pattern of ubiquilin pathology in ALS and FTLD indicates presence of C9ORF72 hexanucleotide expansion
- (2012) Johannes Brettschneider et al. ACTA NEUROPATHOLOGICA
- Bimolecular fluorescence complementation (BiFC): A 5-year update and future perspectives
- (2012) Yutaka Kodama et al. BIOTECHNIQUES
- UBQLN2/ubiquilin 2 mutation and pathology in familial amyotrophic lateral sclerosis
- (2012) Kelly L. Williams et al. NEUROBIOLOGY OF AGING
- Autophagy: Renovation of Cells and Tissues
- (2011) Noboru Mizushima et al. CELL
- Ubiquilin-1 Is a Molecular Chaperone for the Amyloid Precursor Protein
- (2011) Emily S. Stieren et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia
- (2011) Han-Xiang Deng et al. NATURE
- Mammalian Atg18 (WIPI2) localizes to omegasome-anchored phagophores and positively regulates LC3 lipidation
- (2010) Hannah E.J. Polson et al. Autophagy
- An improved bimolecular fluorescence complementation assay with a high signal-to-noise ratio
- (2010) Yutaka Kodama* et al. BIOTECHNIQUES
- Lysosomal Proteolysis and Autophagy Require Presenilin 1 and Are Disrupted by Alzheimer-Related PS1 Mutations
- (2010) Ju-Hyun Lee et al. CELL
- Ubiquilin functions in autophagy and is degraded by chaperone-mediated autophagy
- (2010) C. Rothenberg et al. HUMAN MOLECULAR GENETICS
- Autophagy: cellular and molecular mechanisms
- (2010) Danielle Glick et al. JOURNAL OF PATHOLOGY
- PLIC proteins or ubiquilins regulate autophagy-dependent cell survival during nutrient starvation
- (2009) Elsa-Noah N'Diaye et al. EMBO REPORTS
- The Ubiquitin-like Protein PLIC-2 Is a Negative Regulator of G Protein-coupled Receptor Endocytosis
- (2008) Elsa-Noah N'Diaye et al. MOLECULAR BIOLOGY OF THE CELL
Find Funding. Review Successful Grants.
Explore over 25,000 new funding opportunities and over 6,000,000 successful grants.
ExplorePublish scientific posters with Peeref
Peeref publishes scientific posters from all research disciplines. Our Diamond Open Access policy means free access to content and no publication fees for authors.
Learn More