Cargando…

Reduction in PA28αβ activation in HD mouse brain correlates to increased mHTT aggregation in cell models

Huntington’s disease is an autosomal dominant heritable disorder caused by an expanded CAG trinucleotide repeat at the N-terminus of the Huntingtin (HTT) gene. Lowering the levels of soluble mutant HTT protein prior to aggregation through increased degradation by the proteasome would be a therapeuti...

Descripción completa

Detalles Bibliográficos
Autores principales:	Geijtenbeek, Karlijne W., Janzen, Jolien, Bury, Aleksandra E., Sanz-Sanz, Alicia, Hoebe, Ron A., Bondulich, Marie K., Bates, Gillian P., Reits, Eric A. J., Schipper-Krom, Sabine
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Public Library of Science 2022
Materias:	Research Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9794069/ https://www.ncbi.nlm.nih.gov/pubmed/36574405 http://dx.doi.org/10.1371/journal.pone.0278130

Ejemplares similares

Regulation of Proteasome Activity by (Post-)transcriptional Mechanisms
por: Kors, Suzan, et al.
Publicado: (2019)

C57BL/6 Background Attenuates mHTT Toxicity in the Striatum of YAC128 Mice
por: Back, Michaela K., et al.
Publicado: (2021)

Ubiquitin-modifying enzymes in Huntington’s disease
por: Sap, Karen A., et al.
Publicado: (2023)

Developmental Trajectory of Height, Weight, and BMI in Children and Adolescents at Risk for Huntington’s Disease: Effect of mHTT on Growth
por: Tereshchenko, Alexander, et al.
Publicado: (2020)

PA28αβ: The Enigmatic Magic Ring of the Proteasome?
por: Cascio, Paolo
Publicado: (2014)

Cryo-EM of mammalian PA28αβ-iCP immunoproteasome reveals a distinct mechanism of proteasome activation by PA28αβ
por: Chen, Jinhuan, et al.
Publicado: (2021)

Comparison of mHTT Antibodies in Huntington’s Disease Mouse Models Reveal Specific Binding Profiles and Steady-State Ubiquitin Levels with Disease Development
por: Bayram-Weston, Zubeyde, et al.
Publicado: (2016)

The role of proteasome activators PA28αβ and PA200 in brown adipocyte differentiation and function
por: Koçberber, Zeynep, et al.
Publicado: (2023)

SMAD transcription factors are altered in cell models of HD and regulate HTT expression
por: Bowles, KR, et al.
Publicado: (2017)

Visualizing Proteasome Activity and Intracellular Localization Using Fluorescent Proteins and Activity-Based Probes
por: Schipper-Krom, Sabine, et al.
Publicado: (2019)

Alternative processing of human HTT mRNA with implications for Huntington’s disease therapeutics
por: Fienko, Sandra, et al.
Publicado: (2022)

PA28αβ overexpression enhances learning and memory of female mice without inducing 20S proteasome activity
por: Adelöf, Julia, et al.
Publicado: (2018)

Brain-Derived 11S Regulator (PA28αβ) Promotes Proteasomal Hydrolysis of Elongated Oligoglutamine-Containing Peptides
por: Kriachkov, Viacheslav A., et al.
Publicado: (2023)

Extensive Expression Analysis of Htt Transcripts in Brain Regions from the zQ175 HD Mouse Model Using a QuantiGene Multiplex Assay
por: Papadopoulou, Aikaterini S., et al.
Publicado: (2019)

The Ubiquitin-Proteasome System in Huntington's Disease: Are Proteasomes Impaired, Initiators of Disease, or Coming to the Rescue?
por: Schipper-Krom, Sabine, et al.
Publicado: (2012)

Effector-mediated subversion of proteasome activator (PA)28αβ enhances host defense against Legionella pneumophila under inflammatory and oxidative stress conditions
por: Ngwaga, Tshegofatso, et al.
Publicado: (2023)

HDAC4-Myogenin Axis As an Important Marker of HD-Related Skeletal Muscle Atrophy
por: Mielcarek, Michal, et al.
Publicado: (2015)

Correction: Effector-mediated subversion of proteasome activator (PA)28αβ enhances host defense against Legionella pneumophila under inflammatory and oxidative stress conditions
por: Ngwaga, Tshegofatso, et al.
Publicado: (2023)

The pathogenic exon 1 HTT protein is produced by incomplete splicing in Huntington’s disease patients
por: Neueder, Andreas, et al.
Publicado: (2017)

Identification of a HTT-specific binding motif in DNAJB1 essential for suppression and disaggregation of HTT
por: Ayala Mariscal, S. M., et al.
Publicado: (2022)

HTT-DB: new features and updates
por: Dotto, Bruno Reis, et al.
Publicado: (2018)

A Molecular Docking Study Reveals That Short Peptides Induce Conformational Changes in the Structure of Human Tubulin Isotypes αβI, αβII, αβIII and αβIV
por: Ebenezer, Oluwakemi, et al.
Publicado: (2023)

Proteasome Activators, PA28α and PA28β, Govern Development of Microvascular Injury in Diabetic Nephropathy and Retinopathy
por: Yadranji Aghdam, Saeed, et al.
Publicado: (2016)

PA28γ, an Accomplice to Malignant Cancer
por: Lei, Kexin, et al.
Publicado: (2020)

5-HTT independent effects of fluoxetine on neuroplasticity
por: Levy, Marion J. F., et al.
Publicado: (2019)

HTT haplogroups in Finnish patients with Huntington disease
por: Ylönen, Susanna, et al.
Publicado: (2019)

N-terminal Huntingtin (Htt) phosphorylation is a molecular switch regulating Htt aggregation, helical conformation, internalization, and nuclear targeting
por: DeGuire, Sean M., et al.
Publicado: (2018)

HuR stabilizes HTT mRNA via interacting with its exon 11 in a mutant HTT-dependent manner
por: Zhao, Quan, et al.
Publicado: (2020)

Reply to: Central corneal thickness measurement with Cirrus HD-OCT and Topcon SP-3000P
por: Calvo-Sanz, Jorge A.
Publicado: (2018)

Genesis of the αβ T-cell receptor
por: Dupic, Thomas, et al.
Publicado: (2019)

Proteasome activators, PA28γ and PA200, play indispensable roles in male fertility
por: Huang, Lin, et al.
Publicado: (2016)

HTT2 promotes plant thermotolerance in Brassica rapa
por: Jiang, Jianxia, et al.
Publicado: (2018)

FAN1 modifies Huntington’s disease progression by stabilizing the expanded HTT CAG repeat
por: Goold, Robert, et al.
Publicado: (2019)

Ablation of kynurenine 3-monooxygenase rescues plasma inflammatory cytokine levels in the R6/2 mouse model of Huntington’s disease
por: Bondulich, Marie Katrin, et al.
Publicado: (2021)

PA28γ: New Insights on an Ancient Proteasome Activator
por: Cascio, Paolo
Publicado: (2021)

Loss aversion and 5HTT gene variants in adolescent anxiety
por: Ernst, Monique, et al.
Publicado: (2013)

Rolling Bearing Fault Diagnosis Based on an Improved HTT Transform
por: Pang, Bin, et al.
Publicado: (2018)

Small molecule splicing modifiers with systemic HTT-lowering activity
por: Bhattacharyya, Anuradha, et al.
Publicado: (2021)

The transcriptional landscape of αβ T cell differentiation
por: Mingueneau, Michael, et al.
Publicado: (2013)

Centroid-Based Clustering with αβ-Divergences
por: Sarmiento, Auxiliadora, et al.
Publicado: (2019)

Cannot write session to /tmp/vufind_sessions/sess_dhmt82f1m7pf5rcoc6k5q118b3