Results 81 to 90 of about 16,136 (210)

An Optimal Ubiquitin-Proteasome Pathway in the Nervous System: The Role of Deubiquitinating Enzymes

Frontiers in Molecular Neuroscience, 2014
The Ubiquitin-Proteasome Pathway (UPP), which is critical for normal function in the nervous system and is implicated in various neurological diseases, requires the small modifier protein ubiquitin to accomplish its duty of selectively degrading short ...
Gorica eRistic, Wei-Ling eTsou, Sokol V Todi   +2 more
doaj   +1 more source

Biophysical insights into how surfaces, including lipid membranes, modulate protein aggregation related to neurodegeneration

Frontiers in Neurology, 2013
There are a vast number of neurodegenerative diseases, including Alzheimer’s disease (AD), Parkinson’s disease (PD), and Huntington’s disease (HD), associated with the rearrangement of specific proteins to non-native conformations that promotes ...
Kathleen A Burke, Elizabeth A Yates, Justin eLegleiter   +2 more
doaj   +1 more source

Chemical Chaperones Reduce Aggregate Formation and Cell Death Caused by the Truncated Machado–Joseph Disease Gene Product with an Expanded Polyglutamine Stretch

Neurobiology of Disease, 2002
Machado–Joseph disease/spinocerebellar ataxia-3 (MJD/SCA-3) is an inherited neurodegenerative disorder caused by expansion of the polyglutamine stretch in the MJD gene-encoded protein ataxin-3.
Hideaki Yoshida, Toshihiro Yoshizawa, Futoshi Shibasaki, Shin'ichi Shoji, Ichiro Kanazawa   +4 more
doaj   +1 more source

Quantitative Characterization of Intrinsic Disorder in Polyglutamine: Insights from Analysis Based on Polymer Theories [PDF]

, 2007
Andreas Vitalis, Xiao-Ling Wang, Rohit V. Pappu   +2 more
openalex   +1 more source

The Huntington disease protein accelerates breast tumour development and metastasis through ErbB2/HER2 signalling

EMBO Molecular Medicine, 2013
In Huntington disease (HD), polyglutamine expansion in the huntingtin protein causes specific neuronal death. The consequences of the presence of mutant huntingtin in other tissues are less well understood.
Cristovão Moreira Sousa, John Russel McGuire, Morgane Sonia Thion, David Gentien, Pierre de la Grange, Sophie Tezenas du Montcel, Anne Vincent‐Salomon, Alexandra Durr, Sandrine Humbert   +8 more
doaj   +1 more source

Natural Genetic Variation in Yeast Reveals That NEDD4 Is a Conserved Modifier of Mutant Polyglutamine Aggregation

G3: Genes, Genomes, Genetics, 2018
A feature common to late onset proteinopathic disorders is an accumulation of toxic protein conformers and aggregates in affected tissues. In the search for potential drug targets, many studies used high-throughput screens to find genes that modify the ...
Theodore W. Peters, Christopher S. Nelson, Akos A. Gerencser, Kathleen J. Dumas, Brandon Tavshanjian, Kyu Chul Chang, Gordon J. Lithgow, Robert E. Hughes   +7 more
doaj   +1 more source

Dynamic Imaging by Fluorescence Correlation Spectroscopy Identifies Diverse Populations of Polyglutamine Oligomers Formed in Vivo [PDF]

, 2012
Monica Beam, M. Catarina Silva, Richard I. Morimoto   +2 more
openalex   +1 more source

Enhanced Age-Dependent Motor Impairment in Males of Drosophila melanogaster Modeling Spinocerebellar Ataxia Type 1 Is Linked to Dysregulation of a Matrix Metalloproteinase

Biology
Over the past two decades, Drosophila melanogaster has proven to be successful in modeling the polyglutamine (polyQ) (caused by CAG repeats) family of neurodegenerative disorders, including the faithful recapitulation of pathological features such as ...
Emma M. Palmer, Caleb A. Snoddy, Peyton M. York, Sydney M. Davis, Madelyn F. Hunter, Natraj Krishnan   +5 more
doaj   +1 more source

UBL3 Interacts with PolyQ-Expanded Huntingtin Fragments and Modifies Their Intracellular Sorting

Neurology International
Background/Objectives: UBL3 (Ubiquitin-like 3) is a protein that plays a crucial role in post-translational modifications, particularly in regulating protein transport within small extracellular vesicles. While previous research has predominantly focused
Soho Oyama, Hengsen Zhang, Rafia Ferdous, Yuna Tomochika, Bin Chen, Shuyun Jiang, Md. Shoriful Islam, Md. Mahmudul Hasan, Qing Zhai, A. S. M. Waliullah, Yashuang Ping, Jing Yan, Mst. Afsana Mimi, Chi Zhang, Shuhei Aramaki, Yusuke Takanashi, Tomoaki Kahyo, Yoshio Hashizume, Daita Kaneda, Mitsutoshi Setou   +19 more
doaj   +1 more source

Tadpole-like conformations of huntingtin exon 1 with expanded polyglutamine engenders novel interactions in cells [PDF]

, 2017
Estella A. Newcombe, Kiersten M. Ruff, Ashish Sethi, Angelique R. Ormsby, Yasmin M. Ramdzan, Archa H. Fox, Anthony W. Purcell, Paul R. Gooley, Rohit V. Pappu, Danny M. Hatters   +9 more
openalex   +1 more source