Inhibition of p38 Mitogen–Activated Protein Kinase Ameliorates HAP40 Depletion–Induced Toxicity and Proteasomal Defect in Huntington's Disease Model

ZN Huang, JM Chen, LC Huang, YH Fang… - Molecular neurobiology, 2021 - Springer
… The 26S proteasome activity was monitored by a mutant … core proteasomal peptidase activities
were measured by cell … chymotrypsin-like peptidase activity indirectly. It has been shown …

[HTML][HTML] Targeting the proteostasis network in Huntington's disease

TR Soares, SD Reis, BR Pinho, MR Duchen… - Ageing Research …, 2019 - Elsevier
… are recognized by the 26S proteasome for degradation. The … cellular models (Mitra et al.,
2009). Subsequently, it was proposed that diffuse mHtt might inhibit the proteasome indirectly, …

[HTML][HTML] Proteostasis in Huntington's disease: disease mechanisms and therapeutic opportunities

RJ Harding, Y Tong - Acta Pharmacologica Sinica, 2018 - nature.com
proteasome activity indirectly and in a HD mouse model was … ATP-competitive inhibitors
face issues with toxicity due to … a number of different HD cellular and animal models 125,126 . …

Gene therapy by proteasome activator, PA28γ, improves motor coordination and proteasome function in Huntington's disease YAC128 mice

J Jeon, W Kim, J Jang, O Isacson, H Seo - Neuroscience, 2016 - Elsevier
… system (UPS) are generally inhibited in HD patient’s brain. We previously … that a proteasome
activator (PA), PA28γ enhances proteasome activities and cell survival in in vitro HD model. …

[HTML][HTML] Strategies to investigate ubiquitination in Huntington's disease

KA Sap, EA Reits - Frontiers in Chemistry, 2020 - frontiersin.org
model to study ubiquitination dynamics and the role of the Ubiquitin Proteasome System (UPS)
in protein misfolding diseases… in MG132 proteasome inhibitor concentration in cell culture…

cGMP via PKG activates 26S proteasomes and enhances degradation of proteins, including ones that cause neurodegenerative diseases

JJS VerPlank, SD Tyrkalska… - Proceedings of the …, 2020 - National Acad Sciences
… 5 inhibitors) stimulate proteasome activities and intracellular … larvae models of tauopathies
and Huntingtons disease … Because PKG may activate proteasomes indirectly in cells by …

Proteasome activation as a new therapeutic approach to target proteotoxic disorders

E Njomen, JJ Tepe - Journal of medicinal chemistry, 2019 - ACS Publications
disease (AD), Huntingtons disease (HD), and amyotrophic … in increasing both 20S and 26S
proteasome-mediated proteolysis, … (123) demonstrated that the indirect antioxidant 3H-1,2-…

[HTML][HTML] The proteasome activators Blm10/PA200 enhance the proteasomal degradation of N-terminal huntingtin

A Aladdin, Y Yao, C Yang, G Kahlert, M Ghani, N Király… - Biomolecules, 2020 - mdpi.com
… neither competitive nor noncompetitive inhibitors of 26S and … The expression of Rpn4 is
regulated by the 26S proteasome … has any physiological relevance in Huntingtons disease, we …

[HTML][HTML] Juvenile huntington's disease skin fibroblasts respond with elevated parkin level and increased proteasome activity as a potential mechanism to …

A Aladdin, R Király, P Boto, Z Regdon… - International journal of …, 2019 - mdpi.com
inhibition of the proteasome, while autophagy activity was not significantly affected. That
suggests that parkin might also modulate the activity of the 26S proteasome in our cell model by …

[HTML][HTML] Chemical proteasome inhibition as a novel animal model of inner retinal degeneration in rats

M Kageyama, T Ota, M Sasaoka, O Katsuta… - PLoS …, 2019 - journals.plos.org
… of the tagged protein by the 26S proteasome complex. The … diseases such as Alzheimer,
Parkinson and Huntington … as a direct/indirect proteasome inhibitor under our experimental …