Results 71 to 80 of about 9,105,589 (353)

Strategies to Investigate Ubiquitination in Huntington's Disease

Frontiers in Chemistry, 2020
Many neurodegenerative disorders including Huntington's Disease are hallmarked by intracellular protein aggregates that are decorated by ubiquitin and different ubiquitin ligases and deubiquitinating enzymes. The protein aggregates observed in Huntington'
Karen A. Sap, Eric A. Reits
doaj   +1 more source

Astrocyte Kir4.1 ion channel deficits contribute to neuronal dysfunction in Huntington's disease model mice

Nature Neuroscience, 2014
Huntington's disease (HD) is characterized by striatal medium spiny neuron (MSN) dysfunction, but the underlying mechanisms remain unclear. We explored roles for astrocytes, in which mutant huntingtin is expressed in HD patients and mouse models.
Xiaoping Tong, Y. Ao, G. Faas, Sinifunanya E Nwaobi, Ji Xu, Martin D. Haustein, Mark A. Anderson, I. Módy, M. Olsen, M. Sofroniew, B. Khakh   +10 more
semanticscholar   +1 more source

Suicidality in Huntington's disease

Journal of Affective Disorders, 2012
In Huntington's disease (HD) the risk of suicide is increased. Since suicidality may precede suicide, this study investigates prevalence, clinical associations and predictors of suicidality in HD.Suicidality was investigated in 152 mutation carriers and 56 non-carriers, and was considered present if the score on the item 'suicidal ideation' of the ...
Hubers, A.A.M., Reedeker, N., Giltay, E.J., Roos, R.A.C., Duijn, E. van, Mast, R.C. van der   +5 more
openaire   +6 more sources

Regulation of Neuroinflammation by Microglial DUBA‐IRAK1‐IKKβ Signaling Loop

Advanced Science, EarlyView.
The protein levels of deubiquitinating enzyme A (DUBA) are rapidly upregulated in microglia after activation of Toll‐like receptor 4 (TLR4). In turn, DUBA potentiates TLR4‐induced proinflammatory signal transduction in microglia by stabilizing interleukin‐1 receptor activated kinase 1 (IRAK1) through K48 deubiquitination.
Zhenhu Zhu, Zhongding Li, Kate Lykke Lambertsen, Zijun Cao, Meng Chen, Yanqi Xu, Bettina Hjelm Clausen, Keshuo Jin, Yiting Lu, Fuqi Mei, Xue Du, Kangmin Chen, Huiyuan Bai, Xian Su, Bincheng Zhou, Baohua Liu, Ran Li, Chaoqun Wang, Yuhua Li, Xiang Cai, Dirk Schlüter, Weihong Song, Xu Wang   +22 more
wiley   +1 more source

Mitochondrial Quality Control in Neurodegenerative Diseases: Focus on Parkinson's Disease and Huntington's Disease

Frontiers in Neuroscience, 2018
In recent years, several important advances have been made in our understanding of the pathways that lead to cell dysfunction and death in Parkinson's disease (PD) and Huntington's disease (HD).
Sandra Franco-Iborra, M. Vila, C. Perier
semanticscholar   +1 more source

Mitochondria in Huntington's disease

Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, 2010
Huntington's disease (HD) is an inherited progressive neurodegenerative disorder associated with involuntary abnormal movements (chorea), cognitive deficits and psychiatric disturbances. The disease is caused by an abnormal expansion of a CAG repeat located in exon 1 of the gene encoding the huntingtin protein (Htt) that confers a toxic function to the
Maria Damiano, Laurie Galvan, Laurie Galvan, Nicole Déglon, Nicole Déglon, Emmanuel Brouillet, Emmanuel Brouillet   +6 more
openaire   +5 more sources

Activation of Spinal Astrocyte α2A Adrenoceptors Protects Against Sepsis‐Induced Heart Injury Through Inhibition of GABAergic Neuronal Necroptosis

Advanced Science, EarlyView.
The study in this paper found that activating the α2A receptors of astrocytes in the thoracic spinal cord can reduce the release of inflammatory factors, thereby decreasing the necroptosis of GABAergic neurons and consequently alleviating myocardial injury caused by sepsis.
Ruilin He, Bin Wu, Liuhu Han, Mingde Li, Wenli Guo, Jiajing Fu, Bin Mei, Eric R. Gross, Xuesheng Liu, Yao Lu   +9 more
wiley   +1 more source

m6A Methylation‐Induced Autophagy Impairment by TFEB Regulation in SOD1‐G93A ALS Cell Model

American Journal of Medical Genetics Part B: Neuropsychiatric Genetics, EarlyView.
ABSTRACT We investigate the role of m6A RNA methylation in regulating transcription factor EB (TFEB) and its contribution to mitochondrial autophagy (mitophagy) dysfunction in amyotrophic lateral sclerosis (ALS). ALS cell models were used to analyse mitophagy markers and TFEB expression under METTL3 and TFEB modulation, using RT‐qPCR, Western blot ...
Di An, Yuhao Wu, Jingzhe Han, Pingping Fang, Yi Bu, Guang Ji, Jinliang Deng, Xueqin Song   +7 more
wiley   +1 more source

Establishment of a humanized SCA2 mouse model carrying a CAA disruption preventing CAG repeat expansion in pathogenic genes

Animal Models and Experimental Medicine, EarlyView.
In this study, we established a mouse model in which CAG repeats do not undergo microsatellite instability (MSI) across generations. A humanized ATXN2 cDNA with four CAA interruptions within 73 CAG expansions was inserted into the Rosa26 locus of C57BL/6J mice. At the same time, a 23 CAG control mouse model was also generated.
Yao Zhang, Yufei Li, Lin Zhang, Zhaoqing Li, Keqin Lin, Kai Huang, Zhaoqing Yang, Shaohui Ma, Hao Sun, Xiaochao Zhang   +9 more
wiley   +1 more source

A Drosophila model of prion disease and its metabolic changes in the brain

Animal Models and Experimental Medicine, EarlyView.
We developed a Drosophila model for prion disease, and flies were capable of expressing the hamster prion protein (HaPrP) under the control of the GAL4/UAS system. The model exhibited some characteristics of the disease in mammals and displayed alterations in protein, sphingolipid, and carbohydrate metabolism. Preliminary applications have demonstrated
Dongdong Wang, Zhixin Sun, Pei Wen, Mengyang Zhao, Yuheng He, Fengting Gou, Jingjing Wang, Qing Fan, Xueyuan Li, Tianying Ma, Xiaoyu Wang, Wen Li, Sen Chen, Deming Zhao, Lifeng Yang   +14 more
wiley   +1 more source