Results 101 to 110 of about 9,929 (238)

Suppression of Mutant Protein Expression in SCA3 and SCA1 Mice Using a CAG Repeat-Targeting Antisense Oligonucleotide

Molecular Therapy: Nucleic Acids, 2019
Spinocerebellar ataxia type 3 (SCA3) and type 1 (SCA1) are dominantly inherited neurodegenerative disorders that are currently incurable. Both diseases are caused by a CAG-repeat expansion in exon 10 of the Ataxin-3 and exon 8 of the Ataxin-1 gene ...
Eleni Kourkouta, Rudie Weij, Anchel González-Barriga, Melissa Mulder, Ruurd Verheul, Sieto Bosgra, Bas Groenendaal, Jukka Puoliväli, Jussi Toivanen, Judith C.T. van Deutekom, Nicole A. Datson   +10 more
doaj   +1 more source

Acute optogenetic silencing of orexin/hypocretin neurons induces slow wave sleep in mice [PDF]

, 2011
Orexin/hypocretin neurons have a crucial role in the regulation of sleep and wakefulness. To help determine how these neurons promote wakefulness, we generated transgenic mice in which orexin neurons expressed halorhodopsin (orexin/Halo mice), an orange ...
Boyden, Edward Stuart, Kilduff, Thomas S., Takahashi, Satoru, Tominaga, Makoto, Tsunematsu, Tomomi, Yamanaka, Akihiro   +5 more
core   +1 more source

E3 ligase Praja1 mediates ubiquitination and degradation of microtubule‐associated protein tau

The FEBS Journal, Volume 293, Issue 8, Page 2212-2224, April 2026.
The Praja family of RING‐H2 type E3 ligases is composed of E3 ubiquitin–protein ligases Praja1 and Praja2, which promote the degradation of substrates through the ubiquitin–proteasome system. We show that Praja1, but not its paralogue Praja2, recognizes and ubiquitinates tau protein for proteasomal degradation.
Shiho Aoki, Wataru Onodera, Akihiko Takashima, Kotaro Kawasaki, Kazuki Imadegawa, Hikaru Kurahashi, Mizuho Oishi, Toru Asahi, Yoshiyuki Soeda   +8 more
wiley   +1 more source

Ataxin-3 Is a Multivalent Ligand for the Parkin Ubl Domain

Biochemistry, 2013
The ubiquitin signaling pathway consists of hundreds of enzymes that are tightly regulated for the maintenance of cell homeostasis. Parkin is an E3 ubiquitin ligase responsible for conjugating ubiquitin onto a substrate protein, which itself can be ubiquitinated.
Bai, Jane J., Safadi, Susan S., Mercier, Pascal, Barber, Kathryn R., Shaw, Gary S.   +4 more
openaire   +2 more sources

In vivo suppression of polyglutamine neurotoxicity by C-terminus of Hsp70-interacting protein (CHIP) supports an aggregation model of pathogenesis

Neurobiology of Disease, 2009
Perturbations in neuronal protein homeostasis likely contribute to disease pathogenesis in polyglutamine (polyQ) neurodegenerative disorders. Here we provide evidence that the co-chaperone and ubiquitin ligase, CHIP (C-terminus of Hsp70-interacting ...
Aislinn J. Williams, Tina M. Knutson, Veronica F. Colomer Gould, Henry L. Paulson   +3 more
doaj   +1 more source

Novel candidate blood-based transcriptional biomarkers of Machado-Joseph disease [PDF]

, 2015
BACKGROUND: Machado-Joseph disease (or spinocerebellar ataxia type 3) is a late-onset polyglutamine neurodegenerative disorder caused by a mutation in the ATXN3 gene, which encodes for the ubiquitously expressed protein ataxin-3.
Bettencourt, Bruno, Bettencourt, Conceição, Bruges-Armas, Jácome, Coppola, Giovanni, Gao, Fuying, Geschwind, Daniel, Kay, Teresa, Kazachkova, Nadiya, Lima, Manuela, Maciel, P., Ramos, Amanda, Raposo, Mafalda, Rodrigues, Ana João, Vasconcelos, João   +13 more
core   +2 more sources

The pituitary adenylate cyclase‐activating polypeptide receptor and its splice variants: Unique roles in affective and motivated behavior

Journal of Neuroendocrinology, Volume 38, Issue 4, April 2026.
Abstract Pituitary adenylate cyclase‐activating polypeptide (PACAP) is a pleiotropic neuropeptide with established roles in stress, affective behavior, and motivated behavior. Its primary receptor in the brain, the PACAP type I receptor (PAC1), has multiple variants due to alternative splicing of the gene, and these variants have been found to have ...
Brody A. Carpenter, Jessica R. Barson
wiley   +1 more source

Genome-wide screen for modifiers of ataxin-3 neurodegeneration in Drosophila. [PDF]

PLoS Genetics, 2007
Spinocerebellar ataxia type-3 (SCA3) is among the most common dominantly inherited ataxias, and is one of nine devastating human neurodegenerative diseases caused by the expansion of a CAG repeat encoding glutamine within the gene.
Julide Bilen, Nancy M Bonini
doaj   +1 more source

Disulfide Crosslinking Induces Rapid Degradation of Arc/Arg3.1 via Hsp70‐Mediated Ubiquitin Ligase Pathway

The FASEB Journal, Volume 40, Issue 6, 31 March 2026.
Heat shock induces Arc/Arg3.1 Cys34‐Cys159 disulfide crosslinking, promoting oligomerization and Hsp70‐CHIP‐dependent ubiquitination followed by proteasomal degradation during recovery. Disrupting disulfide formation (C159A) impairs CHIP‐mediated ubiquitination and stabilizes Arc/Arg3.1, whereas HSF1 loss limits inducible Hsp70 and leads to Arc ...
Dami So, In‐Kang Song, Yeon Jung Kim, Yeonjoo Lee, Yeon Seung Park, Hee‐Jung Kim, Kong‐Joo Lee, Eun Joo Song   +7 more
wiley   +1 more source

Caspase‐mediated proteolysis of the polyglutamine disease protein ataxin‐3 [PDF]

Journal of Neurochemistry, 2004
AbstractSpinocerebellar ataxia type‐3, also known as Machado‐Joseph Disease, is one of many inherited neurodegenerative disorders caused by polyglutamine‐encoding CAG repeat expansions in otherwise unrelated disease genes. Polyglutamine disorders are characterized by disease protein misfolding and aggregation; often within the nuclei of affected ...
Berke, S.J.S., Schmied, F.A.F., Brunt, E.R., Ellerby, L.M., Paulson, H.L.   +4 more
openaire   +2 more sources