Results 1 to 10 of about 37,064 (267)

ER stress-induced eIF2-alpha phosphorylation underlies sensitivity of striatal neurons to pathogenic huntingtin. [PDF]

PLoS ONE, 2014
A hallmark of Huntington's disease is the pronounced sensitivity of striatal neurons to polyglutamine-expanded huntingtin expression. Here we show that cultured striatal cells and murine brain striatum have remarkably low levels of phosphorylation of ...
Julia Leitman, Boaz Barak, Ron Benyair, Marina Shenkman, Uri Ashery, F Ulrich Hartl, Gerardo Z Lederkremer   +6 more
doaj   +15 more sources

Huntingtin: A Protein with a Peculiar Solvent Accessible Surface [PDF]

International Journal of Molecular Sciences, 2021
Taking advantage of the last cryogenic electron microscopy structure of human huntingtin, we explored with computational methods its physicochemical properties, focusing on the solvent accessible surface of the protein and highlighting a quite interesting mix of hydrophobic and hydrophilic patterns, with the prevalence of the latter ones.
Giulia Babbi, Castrense Savojardo, Pier Luigi Martelli   +2 more
exaly   +4 more sources

The Ubiquitin-Proteasome Pathway in Huntington's Disease [PDF]

The Scientific World Journal, 2008
The accumulation of mutant protein is a common feature of neurodegenerative disease. In Huntington's disease, a polyglutamine expansion in the huntingtin protein triggers neuronal toxicity.
Siddhartha Mitra, Steven Finkbeiner
doaj   +3 more sources

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

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   +2 more sources

The predominantly HEAT-like motif structure of huntingtin and its association and coincident nuclear entry with dorsal, an NF-kB/Rel/dorsal family transcription factor [PDF]

BMC Neuroscience, 2002
Background Huntington's disease (HD) pathogenesis is due to an expanded polyglutamine tract in huntingtin, but the specificity of neuronal loss compared with other polyglutamine disorders also implies a role for the protein's unknown inherent function ...
Gusella James F, Takano Hiroki
doaj   +3 more sources

Detection of alpha-rod protein repeats using a neural network and application to huntingtin.

PLoS Computational Biology, 2009
A growing number of solved protein structures display an elongated structural domain, denoted here as alpha-rod, composed of stacked pairs of anti-parallel alpha-helices.
Gareth A Palidwor, Sergey Shcherbinin, Matthew R Huska, Tamas Rasko, Ulrich Stelzl, Anup Arumughan, Raphaele Foulle, Pablo Porras, Luis Sanchez-Pulido, Erich E Wanker, Miguel A Andrade-Navarro   +10 more
doaj   +2 more sources

Mutant huntingtin protein decreases with CAG repeat expansion: implications for therapeutics and bioassays. [PDF]

Brain Commun
Landles C, Osborne GF, Phillips J, Canibano-Pico M, Nita IM, Ali N, Bobkov K, Greene JR, Sathasivam K, Bates GP.   +9 more
europepmc   +2 more sources

Quantifying Huntingtin Protein in Human Cerebrospinal Fluid Using a Novel Polyglutamine Length-Independent Assay. [PDF]

J Huntingtons Dis, 2022
Fodale V, Pintauro R, Daldin M, Spiezia MC, Macdonald D, Bresciani A.   +5 more
europepmc   +3 more sources

Mutant Huntingtin Protein Interaction Map Implicates Dysregulation of Multiple Cellular Pathways in Neurodegeneration of Huntington's Disease. [PDF]

J Huntingtons Dis, 2022
Podvin S, Rosenthal SB, Poon W, Wei E, Fisch KM, Hook V.   +5 more
europepmc   +3 more sources

A role of cellular translation regulation associated with toxic Huntingtin protein. [PDF]

Cell Mol Life Sci, 2020
Joag H, Ghatpande V, Desai M, Sarkar M, Raina A, Shinde M, Chitale R, Deo A, Bose T, Majumdar A.   +9 more
europepmc   +3 more sources