Results 31 to 40 of about 27,528 (227)

Metallothioneins and copper metabolism are candidate therapeutic targets in Huntington’s disease [PDF]

, 2010
HD (Huntington's disease) is caused by a polyQ (polyglutamine) expansion in the huntingtin protein, which leads to protein misfolding and aggregation of this protein. Abnormal copper accumulation in the HD brain was first reported more than 15 years ago.
Giorgini, Flaviano, Flaviano Giorgini (58325), Robert Mason (3269031), Sajjad, MU, Hands, Sarah L., M. Umar Sajjad, Wyttenbach, Andreas, Robert Mason, Wyttenbach, A, Mason, Robert, Andreas Wyttenbach, Andreas Wyttenbach (7595339), Umar Sajjad, M., Sarah L. Hands, M. Umar Sajjad (7595336), Sarah L. Hands (7595333), Flaviano Giorgini, Hands, SL, Mason, R; id_orcid   +18 more
core   +1 more source

Huntington's disease: An immune perspective [PDF]

, 2011
Copyright © 2011 Annapurna Nayaketal. This article has been made available through the Brunel Open Access Publishing Fund.Huntington's disease (HD) is a progressive neurodegenerative disorder that is caused by abnormal expansion of CAG trinucleotide ...
Kishore, U, Domenico Marco Bonifati, Ansar, R, Verma, SK, Rafia Ansar, Nayak, A, Sunil K. Verma, Annapurna Nayak, Bonifati, DM, Uday Kishore   +9 more
core   +1 more source

Huntingtin: Here, There, Everywhere!

Journal of Huntington's Disease, 2013
Huntingtin is the protein mutated in Huntington disease, a dominant inherited neurodegenerative disorder. Huntingtin is ubiquitously expressed throughout the body, however its role outside the central nervous system has been overlooked. This review focuses on the peripheral distribution of huntingtin.
Cristovao, Marques Sousa, Sandrine, Humbert   +1 more
openaire   +3 more sources

Small-Molecule Disruptors of Mutant Huntingtin–Calmodulin Protein–Protein Interaction Attenuate Deleterious Effects of Mutant Huntingtin

, 2022
Huntington’s disease is a progressive and lethal neurodegenerative disease caused by an increased CAG repeat mutation in exon 1 of the huntingtin gene (mutant huntingtin).
Kenneth B. Guzmán Rodríguez (13046356), Coral Huntley (13046359), Khushboo Kapadia (13046353), Kevin J. Frankowski (1514284), Ashley E. Trojniak (7581587), Anuradha Roy (630348), Nancy A. Muma (13046362), Nicholas J. Klus (7359803), Peter McDonald (1580623), Jeffrey Aubé (1314141)   +9 more
core   +1 more source

Rescue of aberrant huntingtin palmitoylation ameliorates mutant huntingtin-induced toxicity [PDF]

Neurobiology of Disease, 2021
Abstract Huntington disease (HD) is a neurodegenerative disorder caused by a CAG expansion in the HTT gene that codes for an elongated polyglutamine tract in the huntingtin (HTT) protein.
Fanny L. Lemarié, Nicholas S. Caron, Shaun S. Sanders, Mandi E. Schmidt, Yen T.N. Nguyen, Seunghyun Ko, Xiaohong Xu, Mahmoud A. Pouladi, Dale D.O. Martin, Michael R. Hayden   +9 more
openaire   +3 more sources

Huntingtin associates with the actin cytoskeleton and α-actinin isoforms to influence stimulus dependent morphology changes.

PLoS ONE, 2019
One response of cells to growth factor stimulus involves changes in morphology driven by the actin cytoskeleton and actin associated proteins which regulate functions such as cell adhesion, motility and in neurons, synaptic plasticity.
Adelaide Tousley, Maria Iuliano, Elizabeth Weisman, Ellen Sapp, Heather Richardson, Petr Vodicka, Jonathan Alexander, Neil Aronin, Marian DiFiglia, Kimberly B Kegel-Gleason   +9 more
doaj   +1 more source

Cysteine String Protein Controls Two Routes of Export for Misfolded Huntingtin

Frontiers in Neuroscience, 2022
Extracellular vesicles (EVs) are secreted vesicles of diverse size and cargo that are implicated in the cell-to-cell transmission of disease-causing-proteins in several neurodegenerative diseases.
Desmond Pink, Julien Donnelier, John D. Lewis, John D. Lewis, Janice E. A. Braun   +4 more
doaj   +1 more source

Huntingtin Subcellular Localisation Is Regulated by Kinase Signalling Activity in the StHdhQ111 Model of HD.

PLoS ONE, 2015
Huntington's disease is a neurodegenerative disorder characterised primarily by motor abnormalities, and is caused by an expanded polyglutamine repeat in the huntingtin protein.
Kathryn R Bowles, Simon P Brooks, Stephen B Dunnett, Lesley Jones   +3 more
doaj   +1 more source

Huntingtin’s spherical solenoid structure enables polyglutamine tract-dependent modulation of its structure and function

eLife, 2016
The polyglutamine expansion in huntingtin protein causes Huntington’s disease. Here, we investigated structural and biochemical properties of huntingtin and the effect of the polyglutamine expansion using various biophysical experiments including ...
Ravi Vijayvargia, Raquel Epand, Alexander Leitner, Tae-Yang Jung, Baehyun Shin, Roy Jung, Alejandro Lloret, Randy Singh Atwal, Hyeongseok Lee, Jong-Min Lee, Ruedi Aebersold, Hans Hebert, Ji-Joon Song, Ihn Sik Seong   +13 more
doaj   +1 more source

The localization and interactions of huntingtin [PDF]

Philosophical Transactions of the Royal Society of London. Series B: Biological Sciences, 1999
Huntingtin was localized by using a series of antibodies that detected different areas of the protein from the immediate N–terminus to the C–terminal region of the protein. The more C–terminal antibodies gave a cytoplasmic localization in neurons of the brain in controls and cases of Huntington'sdisease (HD).
openaire   +2 more sources