Results 51 to 60 of about 19,228 (162)

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

Proteostasis in striatal cells and selective neurodegeneration in Huntington’s disease

Frontiers in Cellular Neuroscience, 2014
Selective neuronal loss is a hallmark of neurodegenerative diseases, including Huntington’s disease (HD). Although mutant huntingtin, the protein responsible for Huntington’s disease, is expressed ubiquitously, a subpopulation of neurons in the striatum ...
Julia eMargulis, Julia eMargulis, Steven eFinkbeiner, Steven eFinkbeiner, Steven eFinkbeiner   +4 more
doaj   +1 more source

Reduction of mutant huntingtin accumulation and toxicity by lysosomal cathepsins D and B in neurons

Molecular Neurodegeneration, 2011
Background Huntington's disease is caused by aggregation of mutant huntingtin (mHtt) protein containing more than a 36 polyQ repeat. Upregulation of macroautophagy was suggested as a neuroprotective strategy to degrade mutant huntingtin.
Ouyang Xiaosen, Liang Qiuli, Schneider Lonnie, Zhang Jianhua   +3 more
doaj   +1 more source

Compounds blocking mutant huntingtin toxicity identified using a Huntington's disease neuronal cell model

Neurobiology of Disease, 2005
Neuronal cell death in HD is believed to be largely a dominant cell-autonomous effect of the mutant huntingtin protein. We previously developed an inducible PC12 cell model which expresses an N-terminal huntingtin fragment with an expanded poly Q repeat (
Wenfei Wang, Wenzhen Duan, Shuichi Igarashi, Hokuto Morita, Masayuki Nakamura, Christopher A. Ross   +5 more
doaj   +1 more source

Discovery of sultam-containing small-molecule disruptors of the huntingtin–calmodulin protein–protein interaction

, 2022
N.J. Klus, K. Kapadia, P. McDonald, A. Roy, K.J. Frankowski, N.A. Muma, J. Aubé   +6 more
openalex   +1 more source

Genetic and pharmacological inhibition of calcineurin corrects the BDNF transport defect in Huntington's disease

Molecular Brain, 2009
Background Huntington's disease (HD) is an inherited neurogenerative disease caused by an abnormal expansion of glutamine repeats in the huntingtin protein.
Pineda Jose R, Pardo Raúl, Zala Diana, Yu Hua, Humbert Sandrine, Saudou Frédéric   +5 more
doaj   +1 more source

Delineation of functional subdomains of Huntingtin protein and their interaction with HAP40. [PDF]

Structure, 2023
Alteen MG, Deme JC, Alvarez CP, Loppnau P, Hutchinson A, Seitova A, Chandrasekaran R, Silva Ramos E, Secker C, Alqazzaz M, Wanker EE, Lea SM, Arrowsmith CH, Harding RJ.   +13 more
europepmc   +1 more source

The Cellular and Subcellular Localization of Huntingtin-Associated Protein 1 (HAP1): Comparison with Huntingtin in Rat and Human [PDF]

, 1998
Claire‐Anne Gutekunst, Shihua Li, Hong Yi, Robert J. Ferrante, Xiao‐Jiang Li, Steven M. Hersch   +5 more
openalex   +1 more source

Elucidating the Influence of Lipid Composition on Bilayer Perturbations Induced by the N-terminal Region of the Huntingtin Protein. [PDF]

Biophysica, 2023
Gamage YI, Pan J.
europepmc   +1 more source

Detection of antibodies against the huntingtin protein in human plasma. [PDF]

Cell Mol Life Sci, 2023
Denis HL, Alpaugh M, Alvarez CP, Fenyi A, Barker RA, Chouinard S, Arrowsmith CH, Melki R, Labib R, Harding RJ, Cicchetti F.   +10 more
europepmc   +1 more source