Harnessing chaperone-mediated autophagy for the selective degradation of mutant huntingtin protein

No abstract is available for this paper.

• Publication year

  2010

• Venue

  Nature Biotechnology

• Publication date

  2010-03-01

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1038/nbt.1608PMID 20190739
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Autophagosome formation in mammalian cells

  2010cited by this paper
• Inhibition of Rho Kinases Enhances the Degradation of Mutant Huntingtin*

  2009cited by this paper
• Intrabody Gene Therapy Ameliorates Motor, Cognitive, and Neuropathological Symptoms in Multiple Mouse Models of Huntington's Disease

  2009cited by this paper
• Allele‐specific silencing of mutant Huntington’s disease gene

  2009cited by this paper
• Delivery of the aggregate inhibitor peptide QBP1 into the mouse brain using PTDs and its therapeutic effect on polyglutamine disease mice.

  2009cited by this paper
• The pathogenic mechanisms of polyglutamine diseases and current therapeutic strategies

  2009cited by this paper
• Mutant Huntingtin reduces HSP70 expression through the sequestration of NF‐Y transcription factor

  2008cited by this paper
• The antidepressant sertraline improves the phenotype, promotes neurogenesis and increases BDNF levels in the R6/2 Huntington's disease mouse model.

  2008cited by this paper
• An scFv intrabody against the nonamyloid component of alpha-synuclein reduces intracellular aggregation and toxicity.

  2008cited by this paper
• Blocking acid-sensing ion channel 1 alleviates Huntington's disease pathology via an ubiquitin-proteasome system-dependent mechanism.

  2008cited by this paper
• Intrabodies Binding the Proline-Rich Domains of Mutant Huntingtin Increase Its Turnover and Reduce Neurotoxicity

  2008cited by this paper
• Isolation of a human single chain antibody fragment against oligomeric α-synuclein that inhibits aggregation and prevents α-synuclein induced toxicity

  2007cited by this paper
• Small molecules enhance autophagy and reduce toxicity in Huntington's disease models.

  2007cited by this paper
• Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits

  2007cited by this paper
• A toxic monomeric conformer of the polyglutamine protein

  2007cited by this paper
• Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington’s disease

  2007cited by this paper
• Induction of neostriatal neurogenesis slows disease progression in a transgenic murine model of Huntington disease.

  2007cited by this paper
• Isolation of a human single chain antibody fragment against oligomeric alpha-synuclein that inhibits aggregation and prevents alpha-synuclein-induced toxicity.

  2007cited by this paper
• High-Capacity Adenoviral Vector-Mediated Reduction of Huntingtin Aggregate Load In Vitro and In Vivo

  2007cited by this paper
• Dose ranging and efficacy study of high-dose coenzyme Q10 formulations in Huntington's disease mice.

  2006cited by this paper
• ESET/SETDB1 gene expression and histone H3 (K9) trimethylation in Huntington's disease

  2006cited by this paper
• Tracker Dyes to Probe Mitochondrial Autophagy (Mitophagy) in Rat Hepatocytes

  2006cited by this paper
• Decreased expression of hypothalamic neuropeptides in Huntington disease transgenic mice with expanded polyglutamine‐EGFP fluorescent aggregates

  2005cited by this paper
• Clioquinol down-regulates mutant huntingtin expression in vitro and mitigates pathology in a Huntington's disease mouse model.

  2005cited by this paper
• Intrastriatal rAAV-mediated delivery of anti-huntingtin shRNAs induces partial reversal of disease progression in R6/1 Huntington's disease transgenic mice.

  2005cited by this paper
• The use of the R6 transgenic mouse models of Huntington’s disease in attempts to develop novel therapeutic strategies

  2005cited by this paper
• Semi‐rational engineering of a coral fluorescent protein into an efficient highlighter

  2005cited by this paper
• FGF-2 promotes neurogenesis and neuroprotection and prolongs survival in a transgenic mouse model of Huntington's disease.

  2005cited by this paper
• Clinico-pathological rescue of a model mouse of Huntington's disease by siRNA.

  2005cited by this paper
• RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model.

  2005cited by this paper
• Impaired degradation of mutant alpha-synuclein by chaperone-mediated autophagy.

  2004cited by this paper
• Chemotherapy for the Brain: The Antitumor Antibiotic Mithramycin Prolongs Survival in a Mouse Model of Huntington's Disease

  2004cited by this paper
• Experimental therapeutics in transgenic mouse models of Huntington's disease

  2004cited by this paper
• Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease

  2004cited by this paper
• Impaired Degradation of Mutant α-Synuclein by Chaperone-Mediated Autophagy

  2004cited by this paper
• Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease

  2004cited by this paper
• Prevention of polyglutamine oligomerization and neurodegeneration by the peptide inhibitor QBP1 in Drosophila.

  2003cited by this paper
• Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders

  2003cited by this paper
• Histone Deacetylase Inhibition by Sodium Butyrate Chemotherapy Ameliorates the Neurodegenerative Phenotype in Huntington's Disease Mice

  2003cited by this paper
• Expansion of Polyglutamine Induces the Formation of Quasi-aggregate in the Early Stage of Protein Fibrillization*

  2003cited by this paper
• Pro‐apoptotic protein kinase Cδ is associated with intranuclear inclusions in a transgenic model of Huntington's disease

  2003cited by this paper
• Cathepsin A regulates chaperone‐mediated autophagy through cleavage of the lysosomal receptor

  2003cited by this paper
• Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamine

  2002cited by this paper
• Sp1 and TAFII130 Transcriptional Activity Disrupted in Early Huntington's Disease

  2002cited by this paper
• Aggregation of proteins with expanded glutamine and alanine repeats of the glutamine-rich and asparagine-rich domains of Sup35 and of the amyloid β-peptide of amyloid plaques

  2002cited by this paper
• Autophagosome formation in mammalian cells.

  2002cited by this paper
• Therapeutic Effects of Cystamine in a Murine Model of Huntington's Disease

  2002cited by this paper
• Interference by Huntingtin and Atrophin-1 with CBP-Mediated Transcription Leading to Cellular Toxicity

  2001cited by this paper
• A molecular chaperone complex at the lysosomal membrane is required for protein translocation.

  2001cited by this paper
• Inhibition of Polyglutamine Protein Aggregation and Cell Death by Novel Peptides Identified by Phage Display Screening*

  2000cited by this paper
• Ataxin-3, the MJD1 gene product, interacts with the two human homologs of yeast DNA repair protein RAD23, HHR23A and HHR23B.

  2000cited by this paper
• Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease.

  2000cited by this paper
• Glutamine repeats and neurodegeneration.

  2000cited by this paper
• Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease

  1999cited by this paper
• Preparation of human cDNas encoding expanded polyglutamine repeats.

  1999cited by this paper
• Caspase activation during apoptotic cell death induced by expanded polyglutamine in N2a cells.

  1999cited by this paper
• Transglutaminase aggregates huntingtin into nonamyloidogenic polymers, and its enzymatic activity increases in Huntington's disease brain nuclei.

  1999cited by this paper
• Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation.

  1997cited by this paper
• Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain.

  1997cited by this paper
• A Receptor for the Selective Uptake and Degradation of Proteins by Lysosomes

  1996cited by this paper
• Purification and characterization of lysosomes from Chinese hamster ovary cells.

  1987cited by this paper

• Strategies for targeted protein degradation through lysosomal pathways.

  2026cites this paper
• Expanding the druggable proteome strategies and innovations in targeted protein degradation

  2026cites this paper
• A potential anti-amyloidogenic therapy for type 2 diabetes based on the QBP1 peptide

  2025cites this paper
• Activating autophagy to eliminate toxic protein aggregates with small molecules in neurodegenerative diseases.

  2025cites this paper
• Harnessing the power of anti-amyloidogenic polyglutamine binding peptide 1: A computational and biophysical approach to antimicrobial prediction.

  2025cites this paper
• VEXAS, Chediak–Higashi syndrome and Danon disease: myeloid cell endo-lysosomal pathway dysfunction as a common denominator?

  2025cites this paper
• Autophagy in cancer and protein conformational disorders

  2025cites this paper
• Targeting neurodegenerative disease-associated protein aggregation with proximity-inducing modalities

  2025cites this paper
• Research Advances in Chaperone-Mediated Autophagy (CMA) and CMA-Based Protein Degraders.

  2025cites this paper
• Targeted Degradation Technology Based on the Autophagy‐Lysosomal Pathway: A Promising Strategy for Treating Preeclampsia

  2025cites this paper
• Small molecular fluorescent probes featuring protein-assisted functional amplification for improved biosensing and cancer therapeutics.

  2025cites this paper
• Cell-penetrating proteins and peptides as a promising theragnostic agent for neurodegenerative disorder

  2025cites this paper
• Designing the Proteome with Chemical Tools: Degrons and Beyond

  2025cites this paper
• Deacetylation of HSC70 by SIRT2 promotes chaperone mediated autophagy

  2025cites this paper
• Protein-Based Degraders: From Chemical Biology Tools to Neo-Therapeutics

  2025cites this paper
• Conquering PROTAC molecular design and drugability.

  2025cites this paper
• Bridging structure and selectivity in chaperone-mediated autophagy: towards targeted therapeutics.

  2025cites this paper
• Protein aggregation and therapeutic strategies in SOD1- and TDP-43- linked ALS

  2024cites this paper
• Expanding the horizons of targeted protein degradation: A non-small molecule perspective

  2024cites this paper
• Promising Proteolysis-Targeting Chimera for Mutant p53-R175H

  2024cites this paper
• Autophagy‐Mediated Targeted Protein Degradation

  2024cites this paper
• Chaperone-Mediated Autophagy in Brain Injury: A Double-Edged Sword with Therapeutic Potentials

  2024cites this paper
• Designed Nanodrugs for Ultrasonic Removal of Toxic Polyglutamine Aggregates from Neuron Cells.

  2024cites this paper
• Targeted protein degradation: current molecular targets, localization, and strategies.

  2024cites this paper
• The Role of Chaperone-Mediated Autophagy in Tissue Homeostasis and Disease Pathogenesis

  2024cites this paper
• Targeting chaperone-mediated autophagy in neurodegenerative diseases: mechanisms and therapeutic potential

  2024cites this paper
• An insight into allele-selective approaches to lowering mutant huntingtin protein for Huntington's disease treatment.

  2024cites this paper
• A Novel Lysosome Targeting Chimera for Targeted Protein Degradation via Split-and-Mix Strategy.

  2024cites this paper
• Selective autophagy: a therapeutic target for healthy aging?

  2024cites this paper
• Mitochondria Modulating Therapeutic Approaches in the Management of Huntington’s Disease

  2024cites this paper
• Autophagy as a Therapeutic Target for Multiple Diseases

  2024cites this paper
• Endosome-microautophagy targeting chimera (eMIATAC) for targeted proteins degradation and enhance CAR-T cell anti-tumor therapy

  2024cites this paper
• Targeting EGFR with molecular degraders as a promising strategy to overcome resistance to EGFR inhibitors.

  2024cites this paper
• Autophagy mediated targeting degradation, a promising strategy in drug development.

  2024cites this paper
• Autophagy degradation: a promising dimension in drug discovery for neurodegenerative diseases.

  2024cites this paper
• Unraveling the Puzzle of Therapeutic Peptides: A Promising Frontier in Huntington's Disease Treatment.

  2024cites this paper
• Opportunities for Cellular Rejuvenation in Alzheimer’s Disease: How Epigenetic Reprogramming and Chaperone-Mediated Autophagy Are Enabling Next Generation Therapeutic Approaches

  2023cites this paper
• Biomarker-activated multifunctional lysosome-targeting chimeras mediated selective degradation of extracellular amyloid fibrils

  2023cites this paper
• Recent Advances of PROTACs technology in neurodegenerative diseases

  2023cites this paper
• Enantioselective Degrader for Elimination of Extracellular Aggregation-Prone Proteins hIAPP Associated with Type 2 Diabetes.

  2023cites this paper
• A cell‐penetrating peptide exerts therapeutic effects against ischemic stroke by mediating the lysosomal degradation of sirtuin 5

  2023cites this paper
• Targeted Degradation of Signal Transduction and Activator of Transcription 3 by Chaperone-Mediated Autophagy Targeting Chimeric Nanoplatform.

  2023cites this paper
• LAMP2A, LAMP2B and LAMP2C: similar structures, divergent roles

  2023cites this paper
• Chaperone-mediated autophagy in neurodegenerative diseases: mechanisms and therapy

  2023cites this paper
• Roles of Stress Response in Autophagy Processes and Aging-Related Diseases

  2023cites this paper
• Targeted protein degradation in drug development: Recent advances and future challenges.

  2023cites this paper
• Tethering ATG16L1 or LC3 induces targeted autophagic degradation of protein aggregates and mitochondria

  2023cites this paper
• Peptide-based PROTACs: Current Challenges and Future Perspectives.

  2023cites this paper
• Targeted protein modification as a paradigm shift in drug discovery.

  2023cites this paper
• A Specific Mini‐Intrabody Mediates Lysosome Degradation of Mutant Huntingtin

  2023cites this paper
• Elucidation of Site-Specific Ubiquitination on Chaperones in Response to Mutant Huntingtin

  2023cites this paper
• Neuroprotective effects of chaperone-mediated autophagy in neurodegenerative diseases

  2023cites this paper
• Chaperone‐mediated autophagy: Molecular mechanisms, biological functions, and diseases

  2023cites this paper
• Chaperone-mediated autophagy—mechanisms and disease role

  2022cites this paper
• Mechanisms of RNA and Protein Quality Control and Their Roles in Cellular Senescence and Age-Related Diseases

  2022cites this paper
• Chaperone-Mediated Autophagy in Neurodegenerative Diseases: Molecular Mechanisms and Pharmacological Opportunities

  2022influential citation
• Multidimensional insights into the repeated electromagnetic field stimulation and biosystems interaction in aging and age-related diseases

  2022cites this paper
• Emerging Role of NLRP3 Inflammasome/Pyroptosis in Huntington’s Disease

  2022cites this paper
• The different autophagy degradation pathways and neurodegeneration.

  2022cites this paper
• HSPA8 Is a New Biomarker of Triple Negative Breast Cancer Related to Prognosis and Immune Infiltration

  2022cites this paper
• Emerging Therapies for Huntington’s Disease – Focus on N-Terminal Huntingtin and Huntingtin Exon 1

  2022cites this paper
• BTT_A_270657 141..160

  2022cites this paper
• Targeted protein degraders march towards the clinic for neurodegenerative diseases.

  2022cites this paper
• Targeted protein degradation: mechanisms, strategies and application

  2022cites this paper
• Chaperone-Mediated Autophagy in Neurodegenerative Diseases and Acute Neurological Insults in the Central Nervous System

  2022influential citation
• Hsc70-4 aggravates PolyQ-mediated neurodegeneration by modulating NF-κB mediated immune response in Drosophila

  2022cites this paper
• A Prospective Viewpoint on Neurological Diseases and Their Biomarkers

  2022cites this paper
• Progress and Challenges in Targeted Protein Degradation for Neurodegenerative Disease Therapy.

  2022cites this paper
• Aberrant autophagy in lysosomal storage disorders marked by a lysosomal SNARE protein shortage due to suppression of endocytosis

  2022cites this paper
• Autophagy system as a potential therapeutic target for neurodegenerative diseases.

  2022cites this paper
• Molecular Strategies to Target Protein Aggregation in Huntington’s Disease

  2021cites this paper
• The Role of Chaperone-Mediated Autophagy in Hepatitis C Virus-Induced Pathogenesis

  2021cites this paper
• The Emerging Roles of Autophagy in Human Diseases

  2021cites this paper
• Chaperone-mediated autophagy and disease: Implications for cancer and neurodegeneration.

  2021cites this paper
• Macroautophagy and Mitophagy in Neurodegenerative Disorders: Focus on Therapeutic Interventions

  2021cites this paper
• LAMP2A regulates the loading of proteins into exosomes

  2021cites this paper
• Autophagy in major human diseases

  2021cites this paper
• Developing potent LC3-targeting AUTAC tools for protein degradation with selective autophagy.

  2021cites this paper
• Physiology of cellular demise: Apoptosis, necrosis, and autophagy

  2021cites this paper
• Targeting autophagy in neurodegenerative diseases: From molecular mechanisms to clinical therapeutics

  2021cites this paper
• Targeting Lysosomal Degradation Pathways: New Strategies and Techniques for Drug Discovery.

  2021cites this paper
• Dysfunction of chaperone-mediated autophagy in human diseases

  2021cites this paper
• Basic and Preclinical Research for Personalized Medicine

  2021cites this paper
• Impact of Chaperone-Mediated Autophagy in Brain Aging: Neurodegenerative Diseases and Glioblastoma

  2021cites this paper
• Small molecule probes for targeting autophagy

  2021cites this paper
• Exploring the Role of Autophagy Dysfunction in Neurodegenerative Disorders

  2021cites this paper
• Autophagy and disease: unanswered questions

  2020cites this paper
• Proteome editing using engineered proteins that hijack cellular quality control machinery

  2020cites this paper
• Optimizing intracellular antibodies (intrabodies/nanobodies) to treat neurodegenerative disorders.

  2020cites this paper
• Harnessing the Power of Proteolysis for Targeted Protein Inactivation.

  2020cites this paper
• Understanding the Structure, Toxicity and Inhibition of IAPP at the Nanoscale

  2020cites this paper
• Ubiquitin-interacting motifs of ataxin-3 regulate its polyglutamine toxicity through Hsc70-4-dependent aggregation

  2020cites this paper
• Molecular Chaperones in Neurodegeneration

  2020cites this paper
• Specific Knockdown of α-Synuclein by Peptide-Directed Proteasome Degradation Rescued Its Associated Neurotoxicity.

  2020cites this paper
• HIP1R Expression and Its Association with PD-1 Pathway Blockade Response in Refractory Advanced NonSmall Cell Lung Cancer: A Gene Set Enrichment Analysis

  2020cites this paper
• Mitophagy in the Pathogenesis of Liver Diseases †

  2020cites this paper
• Engineered Extracellular Vesicles/Exosomes as a New Tool against Neurodegenerative Diseases

  2020cites this paper
• Regulation of Cancer Immune Checkpoints: Molecular and Cellular Mechanisms and Therapy

  2020cites this paper
• Recent Progress in PROTACs and Other Chemical Protein Degradation Technologies for the Treatment of Neurodegenerative Disorders.

  2020cites this paper
• Checkpoints Under Traffic Control: From and to Organelles.

  2020cites this paper