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217 Newest Publications about the topic huntington's disease
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A critical role of astrocyte-mediated nuclear factor-{kappa}B-dependent inflammation in Huntington's disease
01-05-2013 | Han-Yun Hsiao; Yu-Chen Chen; Hui-Mei Chen; Pang-Hsien Tu; Yijuang Chern, Human Molecular Genetics, 2013
Huntington's disease (HD) is an autosomal disease caused by a CAG repeat expansion in the huntingtin (HTT) gene. The resultant mutant HTT protein (mHTT) forms aggregates in various types of cells, including neurons and glial cells and preferentially affects brain function. We found that two ...
The potential of carbon‐11 and fluorine‐18 chemistry: illustration through the development of positron emission tomography radioligands targeting the translocator protein 18 kDa
24-04-2013 | Annelaure Damont, Dirk Roeda, Frédéric Dollé, Journal of Labelled Compounds and Radiopharmaceuticals, 2013
The TSPO (translocator protein), also known as the peripheral benzodiazepine receptor, is upregulated in the brain of subjects suffering from neurodegenerative disorders such as Alzheimer's, Parkinson's and Huntington's disease. Moreover, this overexpression has been proved to be linked to ...
Decreased expression of GLT‐1 in the R6/2 model of Huntington's disease does not worsen disease progression
16-04-2013 | Geraldine T. Petr, Laurel A. Schultheis, Kayla C. Hussey, Yan Sun, Janet M. Dubinsky, Chiye Aoki, Paul A. Rosenberg, European Journal of Neuroscience, 2013
Abstract Excitotoxicity is thought to be important in the pathogenesis of Huntington's disease (HD). Glutamate is the predominant excitatory neurotransmitter in the brain, and excess activation of glutamate receptors can cause neuronal dysfunction and death. Glutamate transporters ...
Protein misfolding in the late‐onset neurodegenerative diseases: Common themes and the unique case of amyotrophic lateral sclerosis
18-03-2013 | Vikram Khipple Mulligan, Avijit Chakrabartty, Proteins: Structure, Function, and Bioinformatics, 2013
Abstract Enormous strides have been made in the last hundred years to extend human life expectancy and to combat the major infectious diseases. Today, the major challenges for medical science are age‐related diseases, including cancer, heart disease, lung disease, renal disease, and ...
Elevated NADPH oxidase activity contributes to oxidative stress and cell death in Huntington's disease
15-03-2013 | Antonio Valencia; Ellen Sapp; Jeffrey S. Kimm; Hollis McClory; Patrick B. Reeves; Jonathan Alexander; Kwadwo A. Anso ..., Human Molecular Genetics, 2013
A mutation in the huntingtin (Htt) gene produces mutant Htt and Huntington's disease (HD), a neurodegenerative disorder. HD patients have oxidative damage in the brain, but the causes are unclear. Compared with controls, we found brain levels of NADPH oxidase (NOX) activity, which produces ...
Impaired Mitochondrial Dynamics and Nrf2 Signaling Contribute to Compromised Responses to Oxidative Stress in Striatal Cells Expressing Full-Length Mutant Huntingtin
01-03-2013 | Youngnam N. Jin et al., PLoS ONE, 2013
by Youngnam N. Jin, Yanxun V. Yu, Soner Gundemir, Chulman Jo, Mei Cui, Kim Tieu, Gail V. W. Johnson Huntington disease (HD) is an inherited neurodegenerative disease resulting from an abnormal expansion of polyglutamine in huntingtin (Htt). Compromised oxidative stress defense ...
Defective mitochondrial disulfide relay system, altered mitochondrial morphology and function in Huntington's disease
01-03-2013 | Eleonora Napoli; Sarah Wong; Connie Hung; Catherine Ross-Inta; Prithvi Bomdica; Cecilia Giulivi, Human Molecular Genetics, 2013
A number of studies have been conducted that link mitochondrial dysfunction (MD) to Huntington's disease (HD); however, contradicting results had resulted in a lack of a clear mechanism that links expression of mutant Huntingtin protein and MD. Mouse homozygous (HM) and heterozygous (HT) ...
MSH3 Polymorphisms and Protein Levels Affect CAG Repeat Instability in Huntington's Disease Mice
28-02-2013 | Stéphanie Tomé et al., PLoS Genetics, 2013
by Stéphanie Tomé, Kevin Manley, Jodie P. Simard, Greg W. Clark, Meghan M. Slean, Meera Swami, Peggy F. Shelbourne, Elisabeth R. M. Tillier, Darren G. Monckton, Anne Messer, Christopher E. Pearson Expansions of trinucleotide CAG/CTG repeats in somatic tissues are thought to ...
Doing The Right Thing For One's Children: Deciding Whether To Take The Genetic Test For Huntington's Disease As A Moral Dilemma
25-02-2013 | Jonathan A Smith, Mike Stephenson, Chris Jacobs, Oliver Quarrell, Clinical Genetics, 2013
Abstract This is a qualitative examination of candidates’ decision‐making in relation to the genetic test for Huntington's disease (HD). Semi‐structured interviews were conducted with nine participants who were asked about factors influencing their decision whether to take up predictive ...
Exogenous delivery of chaperonin subunit fragment ApiCCT1 modulates mutant Huntingtin cellular phenotypes [Neuroscience]
19-02-2013 | Emily M. Sontag; Lukasz A. Joachimiak; Zhiqun Tan; Anthony Tomlinson; David E. Housman; Charles G. Glabe; Steven G. ..., Proceedings of the National Academy of Sciences current issue, 2013
Aggregation of misfolded proteins is characteristic of a number of neurodegenerative diseases, including Huntington disease (HD). The CCT/TRiC (chaperonin containing TCP-1/TCP-1 ring) chaperonin complex can inhibit aggregation and cellular toxicity induced by expanded repeat Huntingtin (mHtt) ...
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