Results 91 to 100 of about 6,546,213 (228)

Identification of a Karyopherin β1/β2 Proline-Tyrosine Nuclear Localization Signal in Huntingtin Protein*

Journal of Biological Chemistry, 2012
Background: Huntingtin is a large nuclear protein with no previously identified nuclear localization signal. Results: Huntingtin has a PY-NLS that is recognized by karyopherin β1 and β2.
Carly R. Desmond, R. Atwal, Jianrun Xia, R. Truant   +3 more
semanticscholar   +1 more source

Huntingtin-Associated Protein 1 in Mouse Hypothalamus Stabilizes Glucocorticoid Receptor in Stress Response

Frontiers in Cellular Neuroscience, 2020
Huntingtin-associated protein 1 (Hap1) was initially identified as a brain-enriched protein that binds to the Huntington’s disease protein, huntingtin. Unlike huntingtin that is ubiquitously expressed in the brain, Hap1 is enriched in the brain with the ...
Xingxing Chen, Xingxing Chen, Xingxing Chen, Ning Xin, Yongcheng Pan, Yongcheng Pan, Louyin Zhu, Peng Yin, Qiong Liu, Qiong Liu, Weili Yang, Xingshun Xu, Shihua Li, Xiao-Jiang Li   +13 more
doaj   +1 more source

The Cytotoxicity and Clearance of Mutant Huntingtin and Other Misfolded Proteins

Cells, 2021
Protein misfolding and aggregation are implicated in many neurodegenerative diseases. One of these diseases is Huntington’s, which is caused by increased glutamine-encoding trinucleotide repeats within the Huntingtin gene.
Austin Folger, Yanchang Wang
doaj   +1 more source

An in vitro perspective on the molecular mechanisms underlying mutant huntingtin protein toxicity

Cell Death and Disease, 2012
Huntington’s disease (HD) is a devastating neurodegenerative disorder whose main hallmark is brain atrophy. However, several peripheral organs are considerably affected and their symptoms may, in fact, manifest before those resulting from brain pathology.
G. Cisbani, F. Cicchetti
semanticscholar   +1 more source

The mTOR kinase inhibitor Everolimus decreases S6 kinase phosphorylation but fails to reduce mutant huntingtin levels in brain and is not neuroprotective in the R6/2 mouse model of Huntington's disease

Molecular Neurodegeneration, 2010
Background Huntington's disease (HD) is a progressive neurodegenerative disorder caused by a CAG repeat expansion within the huntingtin gene. Mutant huntingtin protein misfolds and accumulates within neurons where it mediates its toxic effects. Promoting
Frentzel Stefan, Hoyer Daniel, Betschart Claudia, Bleckmann Dorothee, Kama Jibrin A, Dorsey Kate, Chopra Vanita, Connor Teal, Fox Jonathan H, DiFiglia Marian, Paganetti Paolo, Hersch Steven M   +11 more
doaj   +1 more source

Targeting Huntingtin Expression in Patients with Huntington's Disease.

New England Journal of Medicine, 2019
BACKGROUND Huntington's disease is an autosomal-dominant neurodegenerative disease caused by CAG trinucleotide repeat expansion in HTT, resulting in a mutant huntingtin protein.
S. Tabrizi, B. Leavitt, G. Landwehrmeyer, E. Wild, C. Saft, Roger A. Barker, Nick F. Blair, D. Craufurd, J. Priller, H. Rickards, A. Rosser, H. Kordasiewicz, C. Czech, E. Swayze, D. Norris, Tiffany L. Baumann, I. Gerlach, S. Schobel, E. Paz, Anne V Smith, C. Bennett, Roger M. Lane   +21 more
semanticscholar   +1 more source

Mechanisms of copper ion mediated Huntington's disease progression. [PDF]

PLoS ONE, 2007
Huntington's disease (HD) is caused by a dominant polyglutamine expansion within the N-terminus of huntingtin protein and results in oxidative stress, energetic insufficiency and striatal degeneration.
Jonathan H Fox, Jibrin A Kama, Gregory Lieberman, Raman Chopra, Kate Dorsey, Vanita Chopra, Irene Volitakis, Robert A Cherny, Ashley I Bush, Steven Hersch   +9 more
doaj   +1 more source

Rapamycin reduces neuronal mutant huntingtin aggregation and ameliorates locomotor performance in Drosophila

Frontiers in Aging Neuroscience, 2023
Huntington’s disease (HD) is a neurodegenerative disease characterized by movement and cognitive dysfunction. HD is caused by a CAG expansion in exon 1 of the HTT gene that leads to a polyglutamine (PQ) repeat in the huntingtin protein, which aggregates ...
Jonathan R. Roth, Jonathan R. Roth, Ruan Carlos Macedo de Moraes, Brittney P. Xu, Savannah R. Crawley, Malghalara A. Khan, Girish C. Melkani   +6 more
doaj   +1 more source

Fibrillogenesis of Huntingtin and Other Glutamine Containing Proteins [PDF]

, 2012
This chapter focuses on the aggregation of glutamine containing peptides and proteins with an emphasis on huntingtin protein, whose aggregation leads to the development of Huntington's disease. The kinetics that leads to the formation of amyloids, the structure of aggregates of various types and the morphological mechanical properties of amyloid ...
Yuri L, Lyubchenko, Alexey V, Krasnoslobodtsev, Sorin, Luca   +2 more
openaire   +2 more sources

Proteasome activator enhances survival of Huntington's disease neuronal model cells. [PDF]

PLoS ONE, 2007
In patients with Huntington's disease (HD), the proteolytic activity of the ubiquitin proteasome system (UPS) is reduced in the brain and other tissues. The pathological hallmark of HD is the intraneuronal nuclear protein aggregates of mutant huntingtin.
Hyemyung Seo, Kai-Christian Sonntag, Woori Kim, Elena Cattaneo, Ole Isacson   +4 more
doaj   +1 more source