Partial rescue of some features of Huntington Disease in the genetic absence of caspase-6 in YAC128 mice.

No abstract is available for this paper.

• Publication year

  2015

• Venue

  Neurobiology of Disease

• Publication date

  2015-04-01

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1016/j.nbd.2014.12.030PMID 25583186
• 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.

• Autophagy in Huntington disease and huntingtin in autophagy.

  2015cited by this paper
• Measuring apoptosis: caspase inhibitors and activity assays.

  2014cited by this paper
• Distinct Pathways Mediate Axon Degeneration during Apoptosis and Axon-Specific Pruning

  2013cited by this paper
• Huntingtin Acts Non Cell-Autonomously on Hippocampal Neurogenesis and Controls Anxiety-Related Behaviors in Adult Mouse

  2013cited by this paper
• Brain region- and age-dependent dysregulation of p62 and NBR1 in a mouse model of Huntington's disease.

  2013cited by this paper
• Guidelines for the use and interpretation of assays for monitoring autophagy

  2012cited by this paper
• Caspase-6-Resistant Mutant Huntingtin Does not Rescue the Toxic Effects of Caspase-Cleavable Mutant Huntingtin in vivo

  2012cited by this paper
• Caspase-6 Activity in a BACHD Mouse Modulates Steady-State Levels of Mutant Huntingtin Protein But Is Not Necessary for Production of a 586 Amino Acid Proteolytic Fragment

  2012cited by this paper
• Rescue from excitotoxicity and axonal degeneration accompanied by age-dependent behavioral and neuroanatomical alterations in caspase-6-deficient mice.

  2012influential reference
• Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition)

  2012cited by this paper
• Transgenic Mouse Model Expressing the Caspase 6 Fragment of Mutant Huntingtin

  2012cited by this paper
• Caspase-6 does not contribute to the proteolysis of mutant huntingtin in the HdhQ150 knock-in mouse model of Huntington’s disease

  2012cited by this paper
• Targeting the UPR transcription factor XBP1 protects against Huntington's disease through the regulation of FoxO1 and autophagy.

  2012cited by this paper
• Intrinsic cleavage of receptor-interacting protein kinase-1 by caspase-6

  2012cited by this paper
• Involvement of Caspase-6 and Caspase-8 in Neuronal Apoptosis and the Regenerative Failure of Injured Retinal Ganglion Cells

  2011cited by this paper
• Convergent pathogenic pathways in Alzheimer's and Huntington's diseases: shared targets for drug development

  2011cited by this paper
• Autophagy basics

  2011cited by this paper
• Mice lacking caspase-2 are protected from behavioral changes, but not pathology, in the YAC128 model of Huntington disease

  2011cited by this paper
• Neuronal Deletion of Caspase 8 Protects against Brain Injury in Mouse Models of Controlled Cortical Impact and Kainic Acid-Induced Excitotoxicity

  2011cited by this paper
• p62, Ref(2)P and ubiquitinated proteins are conserved markers of neuronal aging, aggregate formation and progressive autophagic defects

  2011cited by this paper
• A Quantitative Method for the Specific Assessment of Caspase-6 Activity in Cell Culture

  2011cited by this paper
• Caspase-6 and neurodegeneration.

  2011cited by this paper
• CARGO RECOGNITION FAILURE IS RESPONSIBLE FOR INEFFICIENT AUTOPHAGY IN HUNTINGTON’S DISEASE

  2010cited by this paper
• A stereological study of MRI and the Cavalieri principle combined for diagnosis and monitoring of brain tumor volume.

  2010cited by this paper
• Huntington disease

  2010cited by this paper
• Genetically engineered mesenchymal stem cells reduce behavioral deficits in the YAC 128 mouse model of Huntington's disease.

  2010cited by this paper
• Early increase in extrasynaptic NMDA receptor signaling and expression contributes to phenotype onset in Huntington's disease mice.

  2010cited by this paper
• Cleavage at the 586 Amino Acid Caspase-6 Site in Mutant huntingtin Influences Caspase-6 Activation In Vivo

  2010cited by this paper
• Phosphorylation of Huntingtin at Ser421 in YAC128 Neurons Is Associated with Protection of YAC128 Neurons from NMDA-Mediated Excitotoxicity and Is Modulated by PP1 and PP2A

  2010cited by this paper
• Early autophagic response in a novel knock-in model of Huntington disease.

  2010cited by this paper
• BDNF Overexpression in the Forebrain Rescues Huntington's Disease Phenotypes in YAC128 Mice

  2010cited by this paper
• Full-length huntingtin levels modulate body weight by influencing insulin-like growth factor 1 expression.

  2010cited by this paper
• Caspase-6 Activation in Familial Alzheimer Disease Brains Carrying Amyloid Precursor Protein or Presenilin I or Presenilin II Mutations

  2009cited by this paper
• Prevention of depressive behaviour in the YAC128 mouse model of Huntington disease by mutation at residue 586 of huntingtin.

  2008cited by this paper
• Overlapping cleavage motif selectivity of caspases: implications for analysis of apoptotic pathways

  2008cited by this paper
• Brain‐derived neurotrophic factor over‐expression in the forebrain ameliorates Huntington’s disease phenotypes in mice

  2008cited by this paper
• Activated caspase-6 and caspase-6-cleaved fragments of huntingtin specifically colocalize in the nucleus.

  2008cited by this paper
• Huntingtin has a membrane association signal that can modulate huntingtin aggregation, nuclear entry and toxicity.

  2007cited by this paper
• Activation of caspase-6 in aging and mild cognitive impairment.

  2007cited by this paper
• Testicular degeneration in Huntington disease.

  2007cited by this paper
• Phenotypic abnormalities in the YAC128 mouse model of Huntington disease are penetrant on multiple genetic backgrounds and modulated by strain.

  2007influential reference
• Body weight is modulated by levels of full-length huntingtin.

  2006cited by this paper
• Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin.

  2006cited by this paper
• Huntingtin fragmentation and increased caspase 3, 8 and 9 activities in lymphoblasts with heterozygous and homozygous Huntington's disease mutation.

  2006cited by this paper
• Levels of mutant huntingtin influence the phenotypic severity of Huntington disease in YAC128 mouse models.

  2006cited by this paper
• Cognitive Dysfunction Precedes Neuropathology and Motor Abnormalities in the YAC128 Mouse Model of Huntington's Disease

  2005cited by this paper
• Huntington Disease

  2005cited by this paper
• Selective degeneration and nuclear localization of mutant huntingtin in the YAC128 mouse model of Huntington disease.

  2005cited by this paper
• Loss of wild-type huntingtin influences motor dysfunction and survival in the YAC128 mouse model of Huntington disease.

  2005cited by this paper
• p62/SQSTM1 forms protein aggregates degraded by autophagy and has a protective effect on huntingtin-induced cell death

  2005cited by this paper
• Caspase-7 Expanded Function and Intrinsic Expression Level Underlies Strain-Specific Brain Phenotype of Caspase-3-Null Mice

  2004cited by this paper
• Specific caspase interactions and amplification are involved in selective neuronal vulnerability in Huntington's disease

  2004cited by this paper
• Inhibition of Calpain Cleavage of Huntingtin Reduces Toxicity

  2004cited by this paper
• Active caspase-6 and caspase-6-cleaved tau in neuropil threads, neuritic plaques, and neurofibrillary tangles of Alzheimer's disease.

  2004cited by this paper
• Q-VD-OPh, a broad spectrum caspase inhibitor with potent antiapoptotic properties

  2003cited by this paper
• Caspase‐8 expression and proteolysis in human brain after severe head injury

  2003cited by this paper
• Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease.

  2003cited by this paper
• Caspase Cleavage of Mutant Huntingtin Precedes Neurodegeneration in Huntington's Disease

  2002cited by this paper
• Activation of caspase-8 in the Alzheimer's disease brain.

  2001cited by this paper
• Compensatory caspase activation: a cautionary tale.

  2001cited by this paper
• Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis

  2001cited by this paper
• Deficiency in caspase-9 or caspase-3 induces compensatory caspase activation

  2000cited by this paper
• Inhibiting Caspase Cleavage of Huntingtin Reduces Toxicity and Aggregate Formation in Neuronal and Nonneuronal Cells*

  2000cited by this paper
• Brain-derived neurotrophic factor in Huntington disease.

  2000cited by this paper
• Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice

  2000cited by this paper
• Caspase Activation Is Required for T Cell Proliferation

  1999cited by this paper
• Caspase Cleavage of Gene Products Associated with Triplet Expansion Disorders Generates Truncated Fragments Containing the Polyglutamine Tract*

  1998cited by this paper
• Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain.

  1997cited by this paper
• A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. The Huntington's Disease Collaborative Research Group.

  1993cited by this paper
• Magnetic resonance imaging (MRI) and model-free estimates of brain volume determined using the Cavalieri principle.

  1991cited by this paper
• Abnormalities of striatal projection neurons and N-methyl-D-aspartate receptors in presymptomatic Huntington's disease.

  1990cited by this paper
• Neuropathological Classification of Huntington's Disease

  1985cited by this paper

• Bioinformatics and next generation sequencing data analysis to identify key genes and pathways influencing in Parkinson's disease

  2025cites this paper
• Using gene or cell therapies to treat Huntington's disease.

  2024cites this paper
• Divalent cations promote huntingtin fibril formation on endoplasmic reticulum derived and model membranes.

  2024cites this paper
• In vivo CRISPR base editing for treatment of Huntington’s disease

  2024cites this paper
• Protective Proteolysis in Huntington’s Disease: Unraveling the Role of Post-Translational Myristoylation of Huntingtin in Autophagy

  2024cites this paper
• Therapeutic approaches targeting aging and cellular senescence in Huntington's disease

  2024cites this paper
• Engaging a Non-catalytic Cysteine Residue Drives Potent and Selective Inhibition of Caspase-6

  2023cites this paper
• Antisense oligonucleotide-mediated disruption of HTT caspase-6 cleavage site ameliorates the phenotype of YAC128 Huntington disease mice.

  2023influential citation
• Full-length huntingtin is palmitoylated at multiple sites and post-translationally myristoylated following caspase-cleavage

  2023cites this paper
• Non-Canonical Roles of Apoptotic Caspases in the Nervous System

  2022cites this paper
• A pathogenic proteolysis–resistant huntingtin isoform induced by an antisense oligonucleotide maintains huntingtin function

  2022cites this paper
• Proteinopathies associated to repeat expansion disorders

  2022cites this paper
• Age-related mitochondrial alterations in brain and skeletal muscle of the YAC128 model of Huntington disease

  2021cites this paper
• Age-related mitochondrial alterations in brain and skeletal muscle of the YAC128 model of Huntington disease

  2021cites this paper
• Linking Huntington disease, brain-derived neurotrophic factor, and depressive-like behaviors

  2021cites this paper
• Insulin-like growth factor 2 (IGF2) protects against Huntington’s disease through the extracellular disposal of protein aggregates

  2020cites this paper
• Newly Identified Function of Caspase-6 in ZBP1-mediated Innate Immune Responses, NLRP3 Inflammasome Activation, PANoptosis, and Host Defense

  2020cites this paper
• Early deficits in olfaction are associated with structural and molecular alterations in the olfactory system of a Huntington disease mouse model.

  2020cites this paper
• Regulation of protein homeostasis in acute and chronic stress

  2019cites this paper
• Targeting the proteostasis network in Huntington’s disease

  2019cites this paper
• Olfactory bulb atrophy and caspase activation observed in the BACHD rat models of Huntington disease.

  2019cites this paper
• The Role of Insulin-Like Growth Factors and Insulin-Like Growth Factor–Binding Proteins in the Nervous System

  2019cites this paper
• Activation of Caspase-6 Is Promoted by a Mutant Huntingtin Fragment and Blocked by an Allosteric Inhibitor Compound.

  2019cites this paper
• Microglial depletion prevents extracellular matrix changes and striatal volume reduction in a model of Huntington's disease.

  2019cites this paper
• The role of Insulin-like Growth Factors (IGFs) and IGF-binding proteins in the nervous system

  2019cites this paper
• Insulin-like growth factor 2 (IGF2) protects against HD through the extracellular disposal of protein aggregates

  2019cites this paper
• Constitutive ablation of caspase-6 reduces the inflammatory response and behavioural changes caused by peripheral pro-inflammatory stimuli

  2018cites this paper
• Differential susceptibility of striatal, hippocampal and cortical neurons to Caspase-6

  2018influential citation
• Preventing mutant huntingtin proteolysis and intermittent fasting promote autophagy in models of Huntington disease

  2018influential citation
• Huntingtin protein: A new option for fixing the Huntington's disease countdown clock

  2018cites this paper
• Activation of caspase‐6 and cleavage of caspase‐6 substrates is an early event in NMDA receptor–mediated excitotoxicity

  2018cites this paper
• Potential of Extracellular Vesicles in Neurodegenerative Diseases: Diagnostic and Therapeutic Indications

  2018cites this paper
• Chapter 4 Porcine Model of Huntington ' s Disease

  2018cites this paper
• Multiple proteolytic events in caspase-6 self-activation impact conformations of discrete structural regions

  2017cites this paper
• Caspase vinyl sulfone small molecule inhibitors prevent axonal degeneration in human neurons and reverse cognitive impairment in Caspase-6-overexpressing mice

  2017cites this paper
• A human huntingtin SNP alters post-translational modification and pathogenic proteolysis of the protein causing Huntington disease

  2017cites this paper
• Identification of a novel caspase cleavage site in huntingtin that regulates mutant huntingtin clearance

  2017influential citation
• Chaperones in Polyglutamine Aggregation: Beyond the Q-Stretch

  2017cites this paper
• Mechanisms underlying neurodegeneration in Huntington disease: applications to novel disease-modifying therapies.

  2017cites this paper
• Global neuroprotection of huntingtin in culture and alterations of cortico-striatal connections in Huntington’s disease culture and mouse models

  2017cites this paper
• The establishment of a flow cytometry system to study HTT regulation and expression

  2017cites this paper
• Feeding schedule and proteolysis regulate autophagic clearance of mutant huntingtin

  2017influential citation
• Caspase-6 Undergoes a Distinct Helix-Strand Interconversion upon Substrate Binding*

  2017cites this paper
• Impact of caspase-6 modulation on Huntington disease phenotypes in the YAC128 mouse model

  2016influential citation
• Laquinimod decreases Bax expression and reduces caspase-6 activation in neurons.

  2016cites this paper
• Laquinimod rescues striatal, cortical and white matter pathology and results in modest behavioural improvements in the YAC128 model of Huntington disease

  2016cites this paper
• Palmitoylation of caspase-6 by HIP14 regulates its activation

  2016cites this paper
• Reprogramming Caspase-7 Specificity by Regio-Specific Mutations and Selection Provides Alternate Solutions for Substrate Recognition.

  2016cites this paper
• Interactome network analysis identifies multiple caspase-6 interactors involved in the pathogenesis of HD.

  2016cites this paper
• Caspase 6 has a protective role in SOD1(G93A) transgenic mice.

  2016cites this paper
• Oxidative metabolism and Ca2+ handling in isolated brain mitochondria and striatal neurons from R6/2 mice, a model of Huntington's disease.

  2016cites this paper
• Identification of Caspase-6 as a New Regulator of Alternatively Activated Macrophages*

  2016cites this paper
• Neuroprotective strategies for NMDAR-mediated excitotoxicity in Huntington’s Disease

  2016cites this paper
• New Views on the Misconstrued: Executioner Caspases and Their Diverse Non-apoptotic Roles.

  2015cites this paper
• A Huntingtin-based peptide inhibitor of caspase-6 provides protection from mutant Huntingtin-induced motor and behavioral deficits.

  2015cites this paper
• Precision autophagy: Will the next wave of selective autophagy markers and specific autophagy inhibitors feed clinical pipelines?

  2015cites this paper