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Structural MRI detects progressive regional brain atrophy and neuroprotective effects in N171-82Q Huntington's disease mouse model

Yong Cheng, Qi Peng, Zhipeng Hou, Manisha Aggarwal, Jiangyang Zhang, Susumu Mori, Christopher A. Ross, Wenzhen Duan
<span title="">2011</span> <i title="Elsevier BV"> <a target="_blank" rel="noopener" href="https://fatcat.wiki/container/sa477uo7lveh7hchpikpixop5u" style="color: black;">NeuroImage</a> </i> &nbsp;
changes in regional brain volumes longitudinally in the N171-82Q HD mouse model in a preclinical trial.  ...  We report here that N171-82Q HD mice exhibit adult-onset and progressive brain atrophy in the striatum and neocortex as well as in whole brain; the progressive atrophy in striatum and neocortex is positively  ...  Duan), NINDS NS16375 (to CAR), NIH EB003543 and NIH ES012665 (to S. Mori), and NIH 65306 (to J. Zhang).  ... 
<span class="external-identifiers"> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.1016/j.neuroimage.2011.02.022">doi:10.1016/j.neuroimage.2011.02.022</a> <a target="_blank" rel="external noopener" href="https://www.ncbi.nlm.nih.gov/pubmed/21320608">pmid:21320608</a> <a target="_blank" rel="external noopener" href="https://pubmed.ncbi.nlm.nih.gov/PMC3372851/">pmcid:PMC3372851</a> <a target="_blank" rel="external noopener" href="https://fatcat.wiki/release/46mi2kxfhrei5jsytxrnduz3au">fatcat:46mi2kxfhrei5jsytxrnduz3au</a> </span>
<a target="_blank" rel="noopener" href="https://web.archive.org/web/20170812105037/http://www.nfomedia.com/static/nfo/1212/resources/HDmouseMRI.pdf" title="fulltext PDF download" data-goatcounter-click="serp-fulltext" data-goatcounter-title="serp-fulltext"> <button class="ui simple right pointing dropdown compact black labeled icon button serp-button"> <i class="icon ia-icon"></i> Web Archive [PDF] <div class="menu fulltext-thumbnail"> <img src="https://blobs.fatcat.wiki/thumbnail/pdf/70/9d/709dcb6252a30691e22aa3fa40700dec2ff1f086.180px.jpg" alt="fulltext thumbnail" loading="lazy"> </div> </button> </a> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.1016/j.neuroimage.2011.02.022"> <button class="ui left aligned compact blue labeled icon button serp-button"> <i class="unlock alternate icon" style="background-color: #fb971f;"></i> elsevier.com </button> </a> <a target="_blank" rel="external noopener" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3372851" title="pubmed link"> <button class="ui compact blue labeled icon button serp-button"> <i class="file alternate outline icon"></i> pubmed.gov </button> </a>

Sirtuin 1 activator SRT2104 protects Huntington's disease mice

Mali Jiang, Jennifer Zheng, Qi Peng, Zhipeng Hou, Jiangyang Zhang, Susumu Mori, James L. Ellis, George P. Vlasuk, Harvey Fries, Vipin Suri, Wenzhen Duan
<span title="2014-10-31">2014</span> <i title="Wiley"> <a target="_blank" rel="noopener" href="https://fatcat.wiki/container/valszhg5dbb5hkurekzuzn2ijq" style="color: black;">Annals of Clinical and Translational Neurology</a> </i> &nbsp;
A pilot effects in N171-82Q Huntington’s disease mouse model.  ...  role in mouse models of Huntington’s disease.  ... 
<span class="external-identifiers"> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.1002/acn3.135">doi:10.1002/acn3.135</a> <a target="_blank" rel="external noopener" href="https://www.ncbi.nlm.nih.gov/pubmed/25574479">pmid:25574479</a> <a target="_blank" rel="external noopener" href="https://pubmed.ncbi.nlm.nih.gov/PMC4284130/">pmcid:PMC4284130</a> <a target="_blank" rel="external noopener" href="https://fatcat.wiki/release/yp2eokjq7zg23md5qmmwu3uive">fatcat:yp2eokjq7zg23md5qmmwu3uive</a> </span>
<a target="_blank" rel="noopener" href="https://web.archive.org/web/20191026200958/http://europepmc.org/backend/ptpmcrender.fcgi?accid=PMC4284130&amp;blobtype=pdf" title="fulltext PDF download" data-goatcounter-click="serp-fulltext" data-goatcounter-title="serp-fulltext"> <button class="ui simple right pointing dropdown compact black labeled icon button serp-button"> <i class="icon ia-icon"></i> Web Archive [PDF] <div class="menu fulltext-thumbnail"> <img src="https://blobs.fatcat.wiki/thumbnail/pdf/04/72/0472e8c3361f30b896524c1402a83a5295e5d2bb.180px.jpg" alt="fulltext thumbnail" loading="lazy"> </div> </button> </a> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.1002/acn3.135"> <button class="ui left aligned compact blue labeled icon button serp-button"> <i class="unlock alternate icon" style="background-color: #fb971f;"></i> wiley.com </button> </a> <a target="_blank" rel="external noopener" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4284130" title="pubmed link"> <button class="ui compact blue labeled icon button serp-button"> <i class="file alternate outline icon"></i> pubmed.gov </button> </a>

Spatiotemporal mapping of brain atrophy in mouse models of Huntington's disease using longitudinal in vivo magnetic resonance imaging

Manisha Aggarwal, Wenzhen Duan, Zhipeng Hou, Neal Rakesh, Qi Peng, Christopher A. Ross, Michael I. Miller, Susumu Mori, Jiangyang Zhang
<span title="2012-02-09">2012</span> <i title="Elsevier BV"> <a target="_blank" rel="noopener" href="https://fatcat.wiki/container/sa477uo7lveh7hchpikpixop5u" style="color: black;">NeuroImage</a> </i> &nbsp;
brain atrophy in the R6/2 and N171-82Q mouse models of HD.  ...  Using an established MRI-based brain atlas and mixed-effects modeling of deformation-based metrics, we report the rates of progression and region-specificity of brain atrophy in the two models.  ...  Duan), NINDS NS16375 (to CAR), NIH EB003543, RR15241, and ES012665 (to S. Mori), and NIHNS065306 (to J. Zhang).  ... 
<span class="external-identifiers"> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.1016/j.neuroimage.2012.01.141">doi:10.1016/j.neuroimage.2012.01.141</a> <a target="_blank" rel="external noopener" href="https://www.ncbi.nlm.nih.gov/pubmed/22342677">pmid:22342677</a> <a target="_blank" rel="external noopener" href="https://pubmed.ncbi.nlm.nih.gov/PMC3321064/">pmcid:PMC3321064</a> <a target="_blank" rel="external noopener" href="https://fatcat.wiki/release/2gxqhc3as5bkvm7af45qzp6izy">fatcat:2gxqhc3as5bkvm7af45qzp6izy</a> </span>
<a target="_blank" rel="noopener" href="https://web.archive.org/web/20200506070602/http://europepmc.org/backend/ptpmcrender.fcgi?accid=PMC3321064&amp;blobtype=pdf" title="fulltext PDF download" data-goatcounter-click="serp-fulltext" data-goatcounter-title="serp-fulltext"> <button class="ui simple right pointing dropdown compact black labeled icon button serp-button"> <i class="icon ia-icon"></i> Web Archive [PDF] <div class="menu fulltext-thumbnail"> <img src="https://blobs.fatcat.wiki/thumbnail/pdf/63/46/6346949a089f7b0bba03b143f5b01b4b233928b5.180px.jpg" alt="fulltext thumbnail" loading="lazy"> </div> </button> </a> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.1016/j.neuroimage.2012.01.141"> <button class="ui left aligned compact blue labeled icon button serp-button"> <i class="unlock alternate icon" style="background-color: #fb971f;"></i> elsevier.com </button> </a> <a target="_blank" rel="external noopener" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3321064" title="pubmed link"> <button class="ui compact blue labeled icon button serp-button"> <i class="file alternate outline icon"></i> pubmed.gov </button> </a>

Neuroprotective role of Sirt1 in mammalian models of Huntington's disease through activation of multiple Sirt1 targets

Mali Jiang, Jiawei Wang, Jinrong Fu, Lin Du, Hyunkyung Jeong, Tim West, Lan Xiang, Qi Peng, Zhipeng Hou, Huan Cai, Tamara Seredenina, Nicolas Arbez (+18 others)
<span title="2011-12-18">2011</span> <i title="Springer Nature"> <a target="_blank" rel="noopener" href="https://fatcat.wiki/container/aowhseoqprea3b5rljynk52q4m" style="color: black;">Nature Medicine</a> </i> &nbsp;
(e,f) Quantification of brain volumes by structural MRI in 16-week-old N171-82Q Huntington's disease mice. Data are mean s.e.m. n = 5.  ...  We detected significant atrophy in the striatum (P < 0.05) and neocortex (P < 0.05) of N171-82Q Huntington's disease mice, which resembled the neuropathological changes seen in human Huntington's disease  ...  We maintained N171-82Q Huntington's disease mice expressing N-terminal fragment HTT with a 82-polyglutamine repeat by breeding Huntington's disease males with C3B6F1 females (Taconic).  ... 
<span class="external-identifiers"> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.1038/nm.2558">doi:10.1038/nm.2558</a> <a target="_blank" rel="external noopener" href="https://www.ncbi.nlm.nih.gov/pubmed/22179319">pmid:22179319</a> <a target="_blank" rel="external noopener" href="https://pubmed.ncbi.nlm.nih.gov/PMC4551453/">pmcid:PMC4551453</a> <a target="_blank" rel="external noopener" href="https://fatcat.wiki/release/cg5e7ma6vbakrkpaohqkqlcih4">fatcat:cg5e7ma6vbakrkpaohqkqlcih4</a> </span>
<a target="_blank" rel="noopener" href="https://web.archive.org/web/20170812105843/http://glennfoundation.org/pdf/Neuroprotective-roles.pdf" title="fulltext PDF download" data-goatcounter-click="serp-fulltext" data-goatcounter-title="serp-fulltext"> <button class="ui simple right pointing dropdown compact black labeled icon button serp-button"> <i class="icon ia-icon"></i> Web Archive [PDF] <div class="menu fulltext-thumbnail"> <img src="https://blobs.fatcat.wiki/thumbnail/pdf/7f/9d/7f9d4dd4cb2962904055a90abf48ef40df7d610b.180px.jpg" alt="fulltext thumbnail" loading="lazy"> </div> </button> </a> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.1038/nm.2558"> <button class="ui left aligned compact blue labeled icon button serp-button"> <i class="external alternate icon"></i> nature.com </button> </a> <a target="_blank" rel="external noopener" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4551453" title="pubmed link"> <button class="ui compact blue labeled icon button serp-button"> <i class="file alternate outline icon"></i> pubmed.gov </button> </a>

Small molecule modulator of protein disulfide isomerase attenuates mutant huntingtin toxicity and inhibits endoplasmic reticulum stress in a mouse model of Huntington's disease

Xiao Zhou, Gang Li, Anna Kaplan, Michael M Gaschler, Xiaoyan Zhang, Zhipeng Hou, Mali Jiang, Roseann Zott, Serge Cremers, Brent R Stockwell, Wenzhen Duan
<span title="2018-02-16">2018</span> <i title="Oxford University Press (OUP)"> <a target="_blank" rel="noopener" href="https://fatcat.wiki/container/b6edj2e2zzf77igiepykq3vzii" style="color: black;">Human Molecular Genetics</a> </i> &nbsp;
In this study, we demonstrated that the PDI is upregulated in the HD human brain, in cell and mouse models.  ...  N171-82Q HD mice.  ...  Juan Troncoso and Olga Pletinikova at the Johns Hopkins Neuropathology for providing the human postmortem brain samples.  ... 
<span class="external-identifiers"> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.1093/hmg/ddy061">doi:10.1093/hmg/ddy061</a> <a target="_blank" rel="external noopener" href="https://www.ncbi.nlm.nih.gov/pubmed/29462355">pmid:29462355</a> <a target="_blank" rel="external noopener" href="https://fatcat.wiki/release/rtbgmoktdvahfmkkmp3zijsdwi">fatcat:rtbgmoktdvahfmkkmp3zijsdwi</a> </span>
<a target="_blank" rel="noopener" href="https://web.archive.org/web/20190303005708/http://pdfs.semanticscholar.org/ac95/ee07af278ccefed6765cf6629ea7ef1e0926.pdf" title="fulltext PDF download" data-goatcounter-click="serp-fulltext" data-goatcounter-title="serp-fulltext"> <button class="ui simple right pointing dropdown compact black labeled icon button serp-button"> <i class="icon ia-icon"></i> Web Archive [PDF] <div class="menu fulltext-thumbnail"> <img src="https://blobs.fatcat.wiki/thumbnail/pdf/ac/95/ac95ee07af278ccefed6765cf6629ea7ef1e0926.180px.jpg" alt="fulltext thumbnail" loading="lazy"> </div> </button> </a> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.1093/hmg/ddy061"> <button class="ui left aligned compact blue labeled icon button serp-button"> <i class="external alternate icon"></i> oup.com </button> </a>

Resting-state functional MRI reveals altered brain connectivity and its correlation with motor dysfunction in a mouse model of Huntington's disease

Qiang Li, Gang Li, Dan Wu, Hanbing Lu, Zhipeng Hou, Christopher A. Ross, Yihong Yang, Jiangyang Zhang, Wenzhen Duan
<span title="">2017</span> <i title="Springer Nature"> <a target="_blank" rel="noopener" href="https://fatcat.wiki/container/tnqhc2x2aneavcd3gx5h7mswhm" style="color: black;">Scientific Reports</a> </i> &nbsp;
In this study, we examined brain regional connectivity and its correlation to brain atrophy, as well as motor function in the 18-week-old N171-82Q HD mice.  ...  Our results suggest that altered brain connectivity detected by resting-state fMRI might serve as an early disease biomarker in HD.  ...  We thank Qi Peng for the technical assistance in taking care of mouse colonies.  ... 
<span class="external-identifiers"> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.1038/s41598-017-17026-5">doi:10.1038/s41598-017-17026-5</a> <a target="_blank" rel="external noopener" href="https://www.ncbi.nlm.nih.gov/pubmed/29196686">pmid:29196686</a> <a target="_blank" rel="external noopener" href="https://pubmed.ncbi.nlm.nih.gov/PMC5711837/">pmcid:PMC5711837</a> <a target="_blank" rel="external noopener" href="https://fatcat.wiki/release/tjasveek2fcrphlawqz4pjglfu">fatcat:tjasveek2fcrphlawqz4pjglfu</a> </span>
<a target="_blank" rel="noopener" href="https://web.archive.org/web/20191021202644/http://europepmc.org/backend/ptpmcrender.fcgi?accid=PMC5711837&amp;blobtype=pdf" title="fulltext PDF download" data-goatcounter-click="serp-fulltext" data-goatcounter-title="serp-fulltext"> <button class="ui simple right pointing dropdown compact black labeled icon button serp-button"> <i class="icon ia-icon"></i> Web Archive [PDF] <div class="menu fulltext-thumbnail"> <img src="https://blobs.fatcat.wiki/thumbnail/pdf/44/3f/443f6f2d998d7b3d2709c29f5ae1d8993eb7d324.180px.jpg" alt="fulltext thumbnail" loading="lazy"> </div> </button> </a> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.1038/s41598-017-17026-5"> <button class="ui left aligned compact blue labeled icon button serp-button"> <i class="unlock alternate icon" style="background-color: #fb971f;"></i> Publisher / doi.org </button> </a> <a target="_blank" rel="external noopener" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5711837" title="pubmed link"> <button class="ui compact blue labeled icon button serp-button"> <i class="file alternate outline icon"></i> pubmed.gov </button> </a>

Rhes Suppression Enhances Disease Phenotypes in Huntington's Disease Mice

John H. Lee, Matthew J. Sowada, Ryan L. Boudreau, Andrea M. Aerts, Daniel R. Thedens, Peg Nopoulos, Beverly L. Davidson
<span title="">2014</span> <i title="IOS Press"> <a target="_blank" rel="noopener" href="https://fatcat.wiki/container/dwppn2jr4nfzpbnamyl42mz75q" style="color: black;">Journal of Huntington&#39;s Disease</a> </i> &nbsp;
While Rhes suppression was tolerated in wildtype mice, it failed to improve rotarod function in two distinct HD mouse models.  ...  In Huntington's disease (HD) mutant HTT is ubiquitously expressed yet the striatum undergoes profound early degeneration.  ...  ACKNOWLEDGMENTS The work was supported by Carver College Collaborative grant, NIH grants NS050568-03 (to PN), NIH grant NS50210 and NS076631 (to BLD) and the Roy J Carver Trust.  ... 
<span class="external-identifiers"> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.3233/jhd-140094">doi:10.3233/jhd-140094</a> <a target="_blank" rel="external noopener" href="https://www.ncbi.nlm.nih.gov/pubmed/25062765">pmid:25062765</a> <a target="_blank" rel="external noopener" href="https://pubmed.ncbi.nlm.nih.gov/PMC4139702/">pmcid:PMC4139702</a> <a target="_blank" rel="external noopener" href="https://fatcat.wiki/release/setrnrqlunhsxmopvc5hwrxvoe">fatcat:setrnrqlunhsxmopvc5hwrxvoe</a> </span>
<a target="_blank" rel="noopener" href="https://web.archive.org/web/20191208192001/https://content.iospress.com:443/download/journal-of-huntingtons-disease/jhd140094?id=journal-of-huntingtons-disease%2Fjhd140094" title="fulltext PDF download" data-goatcounter-click="serp-fulltext" data-goatcounter-title="serp-fulltext"> <button class="ui simple right pointing dropdown compact black labeled icon button serp-button"> <i class="icon ia-icon"></i> Web Archive [PDF] <div class="menu fulltext-thumbnail"> <img src="https://blobs.fatcat.wiki/thumbnail/pdf/c6/31/c631b08cf16b35332388060f5eeb5d9ede9745b0.180px.jpg" alt="fulltext thumbnail" loading="lazy"> </div> </button> </a> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.3233/jhd-140094"> <button class="ui left aligned compact blue labeled icon button serp-button"> <i class="unlock alternate icon" style="background-color: #fb971f;"></i> Publisher / doi.org </button> </a> <a target="_blank" rel="external noopener" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4139702" title="pubmed link"> <button class="ui compact blue labeled icon button serp-button"> <i class="file alternate outline icon"></i> pubmed.gov </button> </a>

Neuroprotective Effects of Psychotropic Drugs in Huntington's Disease

Edward Lauterbach
<span title="2013-11-15">2013</span> <i title="MDPI AG"> <a target="_blank" rel="noopener" href="https://fatcat.wiki/container/3loumxx7kzamnlu4h6x3xoz6ay" style="color: black;">International Journal of Molecular Sciences</a> </i> &nbsp;
In HD preclinical models, while no psychotropic has convincingly affected huntingtin gene, HD modifying gene, or huntingtin protein expression, psychotropic neuroprotective effects include upregulated  ...  Most preclinical findings await replication and their limitations are reviewed. The most promising findings involve replicated striatal neuroprotection and phenotypic disease OPEN ACCESS Int. J. Mol.  ...  effects in Huntington's disease models.  ... 
<span class="external-identifiers"> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.3390/ijms141122558">doi:10.3390/ijms141122558</a> <a target="_blank" rel="external noopener" href="https://www.ncbi.nlm.nih.gov/pubmed/24248060">pmid:24248060</a> <a target="_blank" rel="external noopener" href="https://pubmed.ncbi.nlm.nih.gov/PMC3856079/">pmcid:PMC3856079</a> <a target="_blank" rel="external noopener" href="https://fatcat.wiki/release/4jteldp57fcgjcw4rsqs455cyq">fatcat:4jteldp57fcgjcw4rsqs455cyq</a> </span>
<a target="_blank" rel="noopener" href="https://web.archive.org/web/20151203100044/http://www.mdpi.com/1422-0067/14/11/22558/pdf" title="fulltext PDF download" data-goatcounter-click="serp-fulltext" data-goatcounter-title="serp-fulltext"> <button class="ui simple right pointing dropdown compact black labeled icon button serp-button"> <i class="icon ia-icon"></i> Web Archive [PDF] <div class="menu fulltext-thumbnail"> <img src="https://blobs.fatcat.wiki/thumbnail/pdf/a1/9a/a19a785f62bfc24beeaf686c38ef61e3307f2d2d.180px.jpg" alt="fulltext thumbnail" loading="lazy"> </div> </button> </a> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.3390/ijms141122558"> <button class="ui left aligned compact blue labeled icon button serp-button"> <i class="unlock alternate icon" style="background-color: #fb971f;"></i> mdpi.com </button> </a> <a target="_blank" rel="external noopener" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3856079" title="pubmed link"> <button class="ui compact blue labeled icon button serp-button"> <i class="file alternate outline icon"></i> pubmed.gov </button> </a>

The Role of Hypothalamic Pathology for Non-Motor Features of Huntington's Disease

Rachel Y. Cheong, Sanaz Gabery, Åsa Petersén
<span title="2019-09-26">2019</span> <i title="IOS Press"> <a target="_blank" rel="noopener" href="https://fatcat.wiki/container/dwppn2jr4nfzpbnamyl42mz75q" style="color: black;">Journal of Huntington&#39;s Disease</a> </i> &nbsp;
The hypothalamus and the limbic system are important brain regions that regulate emotion, social cognition, sleep and metabolism.  ...  Huntington's disease (HD) is a fatal genetic neurodegenerative disorder.  ...  Neuroprotective effects of SIRT1 inhibitors have been shown in drosophila, mammalian cells and mouse models of HD [69] .  ... 
<span class="external-identifiers"> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.3233/jhd-190372">doi:10.3233/jhd-190372</a> <a target="_blank" rel="external noopener" href="https://www.ncbi.nlm.nih.gov/pubmed/31594240">pmid:31594240</a> <a target="_blank" rel="external noopener" href="https://pubmed.ncbi.nlm.nih.gov/PMC6839491/">pmcid:PMC6839491</a> <a target="_blank" rel="external noopener" href="https://fatcat.wiki/release/6xipcdclpncfpjl4avd34tfeh4">fatcat:6xipcdclpncfpjl4avd34tfeh4</a> </span>
<a target="_blank" rel="noopener" href="https://web.archive.org/web/20200306150221/https://content.iospress.com/download/journal-of-huntingtons-disease/jhd190372?id=journal-of-huntingtons-disease%2Fjhd190372" title="fulltext PDF download" data-goatcounter-click="serp-fulltext" data-goatcounter-title="serp-fulltext"> <button class="ui simple right pointing dropdown compact black labeled icon button serp-button"> <i class="icon ia-icon"></i> Web Archive [PDF] <div class="menu fulltext-thumbnail"> <img src="https://blobs.fatcat.wiki/thumbnail/pdf/0f/8f/0f8f168d9ccbd39a7d9dcd95a9a1b3f176f7d2c6.180px.jpg" alt="fulltext thumbnail" loading="lazy"> </div> </button> </a> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.3233/jhd-190372"> <button class="ui left aligned compact blue labeled icon button serp-button"> <i class="external alternate icon"></i> Publisher / doi.org </button> </a> <a target="_blank" rel="external noopener" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6839491" title="pubmed link"> <button class="ui compact blue labeled icon button serp-button"> <i class="file alternate outline icon"></i> pubmed.gov </button> </a>

PGC-1α, mitochondrial dysfunction, and Huntington's disease

Ashu Johri, Abhishek Chandra, M. Flint Beal
<span title="">2013</span> <i title="Elsevier BV"> <a target="_blank" rel="noopener" href="https://fatcat.wiki/container/6slq4g5rujaijcqpb7lq3egq5q" style="color: black;">Free Radical Biology &amp; Medicine</a> </i> &nbsp;
There is strong evidence that mitochondrial dysfunction results in neurodegeneration and may contribute to the pathogenesis of Huntington's disease (HD).  ...  Here we have attempted to discuss in a nutshell, the key findings on the role of PGC-1α in mitochondrial dysfunction in HD and its potential as a therapeutic target to cure HD.  ...  Pathologically, there is a preferential and progressive loss of the medium spiny neurons (MSNs) in the striatum, as well as cortical atrophy, and degeneration of other brain regions later in the disease  ... 
<span class="external-identifiers"> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.1016/j.freeradbiomed.2013.04.016">doi:10.1016/j.freeradbiomed.2013.04.016</a> <a target="_blank" rel="external noopener" href="https://www.ncbi.nlm.nih.gov/pubmed/23602910">pmid:23602910</a> <a target="_blank" rel="external noopener" href="https://pubmed.ncbi.nlm.nih.gov/PMC3722269/">pmcid:PMC3722269</a> <a target="_blank" rel="external noopener" href="https://fatcat.wiki/release/ewnmj5fc45glbauh2h35c2ep5u">fatcat:ewnmj5fc45glbauh2h35c2ep5u</a> </span>
<a target="_blank" rel="noopener" href="https://web.archive.org/web/20200206040530/http://europepmc.org/backend/ptpmcrender.fcgi?accid=PMC3722269&amp;blobtype=pdf" title="fulltext PDF download" data-goatcounter-click="serp-fulltext" data-goatcounter-title="serp-fulltext"> <button class="ui simple right pointing dropdown compact black labeled icon button serp-button"> <i class="icon ia-icon"></i> Web Archive [PDF] <div class="menu fulltext-thumbnail"> <img src="https://blobs.fatcat.wiki/thumbnail/pdf/59/d3/59d3b644cb60850453cbd78e466f66c9785673f3.180px.jpg" alt="fulltext thumbnail" loading="lazy"> </div> </button> </a> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.1016/j.freeradbiomed.2013.04.016"> <button class="ui left aligned compact blue labeled icon button serp-button"> <i class="external alternate icon"></i> elsevier.com </button> </a> <a target="_blank" rel="external noopener" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3722269" title="pubmed link"> <button class="ui compact blue labeled icon button serp-button"> <i class="file alternate outline icon"></i> pubmed.gov </button> </a>

Mechanisms of Copper Ion Mediated Huntington's Disease Progression

Jonathan H. Fox, Jibrin A. Kama, Gregory Lieberman, Raman Chopra, Kate Dorsey, Vanita Chopra, Irene Volitakis, Robert A. Cherny, Ashley I. Bush, Steven Hersch, Katrina Gwinn-Hardy
<span title="2007-03-28">2007</span> <i title="Public Library of Science (PLoS)"> <a target="_blank" rel="noopener" href="https://fatcat.wiki/container/s3gm7274mfe6fcs7e3jterqlri" style="color: black;">PLoS ONE</a> </i> &nbsp;
Huntington's disease (HD) is caused by a dominant polyglutamine expansion within the N-terminus of huntingtin protein and results in oxidative stress, energetic insufficiency and striatal degeneration.  ...  Further, copper promoted and metal chelation inhibited aggregation of cellfree huntingtin. We found decreased LDH activity, but not protein, and increased lactate levels in HD transgenic mouse brain.  ...  Wilma Wasco and Marian Difiglia for the anti-APLP1 antibody and AB1 antibodies and Dr. Jack Rogers for helpful suggestions. We also thank Dr.  ... 
<span class="external-identifiers"> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.1371/journal.pone.0000334">doi:10.1371/journal.pone.0000334</a> <a target="_blank" rel="external noopener" href="https://www.ncbi.nlm.nih.gov/pubmed/17396163">pmid:17396163</a> <a target="_blank" rel="external noopener" href="https://pubmed.ncbi.nlm.nih.gov/PMC1828629/">pmcid:PMC1828629</a> <a target="_blank" rel="external noopener" href="https://fatcat.wiki/release/c4ugtjuggrb6ddx47kroyccdpe">fatcat:c4ugtjuggrb6ddx47kroyccdpe</a> </span>
<a target="_blank" rel="noopener" href="https://web.archive.org/web/20170921211601/http://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0000334&amp;type=printable" title="fulltext PDF download" data-goatcounter-click="serp-fulltext" data-goatcounter-title="serp-fulltext"> <button class="ui simple right pointing dropdown compact black labeled icon button serp-button"> <i class="icon ia-icon"></i> Web Archive [PDF] <div class="menu fulltext-thumbnail"> <img src="https://blobs.fatcat.wiki/thumbnail/pdf/7e/f5/7ef5e7a2c58a5e89951f3690f804e8ce9cebcd23.180px.jpg" alt="fulltext thumbnail" loading="lazy"> </div> </button> </a> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.1371/journal.pone.0000334"> <button class="ui left aligned compact blue labeled icon button serp-button"> <i class="unlock alternate icon" style="background-color: #fb971f;"></i> plos.org </button> </a> <a target="_blank" rel="external noopener" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1828629" title="pubmed link"> <button class="ui compact blue labeled icon button serp-button"> <i class="file alternate outline icon"></i> pubmed.gov </button> </a>

Mouse models of Huntington's disease and methodological considerations for therapeutic trials

Robert J. Ferrante
<span title="">2009</span> <i title="Elsevier BV"> <a target="_blank" rel="noopener" href="https://fatcat.wiki/container/2hsw6h7ajnhunhtep2vw5jb2qe" style="color: black;">Biochimica et Biophysica Acta - Molecular Basis of Disease</a> </i> &nbsp;
A review of the current state of HD mouse models and their successes in elucidating disease pathogenesis are discussed.  ...  Huntington's disease (HD) is an autosomal dominant, progressive, and fatal neurodegenerative disorder caused by an expanded polyglutamine cytosine-adenine-guanine repeat in the gene coding for the protein  ...  Acknowledgements This work was supported by National Institutes of Health Grants NS045242, NS045806, and NS058793, and by the Veterans Administration.  ... 
<span class="external-identifiers"> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.1016/j.bbadis.2009.04.001">doi:10.1016/j.bbadis.2009.04.001</a> <a target="_blank" rel="external noopener" href="https://www.ncbi.nlm.nih.gov/pubmed/19362590">pmid:19362590</a> <a target="_blank" rel="external noopener" href="https://pubmed.ncbi.nlm.nih.gov/PMC2693467/">pmcid:PMC2693467</a> <a target="_blank" rel="external noopener" href="https://fatcat.wiki/release/36d3dfsr5vbmlic2ugdxho6sby">fatcat:36d3dfsr5vbmlic2ugdxho6sby</a> </span>
<a target="_blank" rel="noopener" href="https://web.archive.org/web/20190413222731/https://core.ac.uk/download/pdf/82314291.pdf" title="fulltext PDF download" data-goatcounter-click="serp-fulltext" data-goatcounter-title="serp-fulltext"> <button class="ui simple right pointing dropdown compact black labeled icon button serp-button"> <i class="icon ia-icon"></i> Web Archive [PDF] <div class="menu fulltext-thumbnail"> <img src="https://blobs.fatcat.wiki/thumbnail/pdf/ac/61/ac61d70b3b59eace9fb8af119f06bbba4351e840.180px.jpg" alt="fulltext thumbnail" loading="lazy"> </div> </button> </a> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.1016/j.bbadis.2009.04.001"> <button class="ui left aligned compact blue labeled icon button serp-button"> <i class="external alternate icon"></i> elsevier.com </button> </a> <a target="_blank" rel="external noopener" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2693467" title="pubmed link"> <button class="ui compact blue labeled icon button serp-button"> <i class="file alternate outline icon"></i> pubmed.gov </button> </a>

Mitochondria in Huntington's disease

Maria Damiano, Laurie Galvan, Nicole Déglon, Emmanuel Brouillet
<span title="">2010</span> <i title="Elsevier BV"> <a target="_blank" rel="noopener" href="https://fatcat.wiki/container/2hsw6h7ajnhunhtep2vw5jb2qe" style="color: black;">Biochimica et Biophysica Acta - Molecular Basis of Disease</a> </i> &nbsp;
Huntington's disease (HD) is an inherited progressive neurodegenerative disorder associated with involuntary abnormal movements (chorea), cognitive deficits and psychiatric disturbances.  ...  Studies investigating the toxic effects of mutant Htt in cell culture or animal models reveal mitochondrial changes including reduction of Ca 2+ buffering capacity, loss of membrane potential, and decreased  ...  Acknowledgements L.G. is supported for her PhD training program by Neuropole de Recherche Francilien (NeRF), Région Ile-de-France.  ... 
<span class="external-identifiers"> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.1016/j.bbadis.2009.07.012">doi:10.1016/j.bbadis.2009.07.012</a> <a target="_blank" rel="external noopener" href="https://www.ncbi.nlm.nih.gov/pubmed/19682570">pmid:19682570</a> <a target="_blank" rel="external noopener" href="https://fatcat.wiki/release/u5bvfqqnqbcf3ehg6pf3fnzk44">fatcat:u5bvfqqnqbcf3ehg6pf3fnzk44</a> </span>
<a target="_blank" rel="noopener" href="https://web.archive.org/web/20170926082452/http://publisher-connector.core.ac.uk/resourcesync/data/elsevier/pdf/3a6/aHR0cDovL2FwaS5lbHNldmllci5jb20vY29udGVudC9hcnRpY2xlL3BpaS9zMDkyNTQ0MzkwOTAwMTY1Mw%3D%3D.pdf" title="fulltext PDF download" data-goatcounter-click="serp-fulltext" data-goatcounter-title="serp-fulltext"> <button class="ui simple right pointing dropdown compact black labeled icon button serp-button"> <i class="icon ia-icon"></i> Web Archive [PDF] <div class="menu fulltext-thumbnail"> <img src="https://blobs.fatcat.wiki/thumbnail/pdf/28/0d/280d925b7eddf9ad7fc65b45f7d8f7ed3f2a4182.180px.jpg" alt="fulltext thumbnail" loading="lazy"> </div> </button> </a> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.1016/j.bbadis.2009.07.012"> <button class="ui left aligned compact blue labeled icon button serp-button"> <i class="external alternate icon"></i> elsevier.com </button> </a>

Transgenic animal models for study of the pathogenesis of Huntington's disease and therapy

Chang RB, Liu XD, Li SH, Li XJ
<span title="">2015</span> <i title="Dove Medical Press"> <a target="_blank" rel="noopener" href="https://fatcat.wiki/container/fiqgu2g3kfawzan3zs5ni7lrfy" style="color: black;">Drug Design, Development and Therapy</a> </i> &nbsp;
Keywords: transgenic animal models, Huntington's disease, pathogenesis, therapy  ...  expansion in the N-terminal regions of huntingtin.  ...  Transgenic HD mice, such as R6/2 and N171-82Q, develop severe phenotypes with rapid disease progression.  ... 
<span class="external-identifiers"> <a target="_blank" rel="external noopener" href="https://doaj.org/article/2e509edc2b9e439cb449a9ebdaa3a282">doaj:2e509edc2b9e439cb449a9ebdaa3a282</a> <a target="_blank" rel="external noopener" href="https://fatcat.wiki/release/6esv5bachrhwpfloui7efb4cdm">fatcat:6esv5bachrhwpfloui7efb4cdm</a> </span>
<a target="_blank" rel="noopener" href="https://web.archive.org/web/20220224192525/https://www.dovepress.com/transgenic-animal-models-for-study-of-the-pathogenesis-of-huntingtonrs-peer-reviewed-fulltext-article-DDDT" title="fulltext access" data-goatcounter-click="serp-fulltext" data-goatcounter-title="serp-fulltext"> <button class="ui simple right pointing dropdown compact black labeled icon button serp-button"> <i class="icon ia-icon"></i> Web Archive [HTML] </button> </a>

Prospects for neuroprotective therapies in prodromal Huntington's disease

Abhishek Chandra, Ashu Johri, M. Flint Beal
<span title="2014-02-26">2014</span> <i title="Wiley"> <a target="_blank" rel="noopener" href="https://fatcat.wiki/container/lbpdbfzorrcddi4zhcvfx2arnq" style="color: black;">Movement Disorders</a> </i> &nbsp;
Huntington's disease (HD) is a prototypical dominantly inherited neurodegenerative disorder which is characterized by progressive cognitive deterioration, psychiatric disturbances and a movement disorder  ...  We review both the earliest detectable clinical and laboratory manifestations of HD, as well as potential neuroprotective therapies which could be utilized in presymptomatic HD.  ...  glucose uptake. 44 We carried out studies of glucose utilization in several HD transgenic mouse models including N171-82Q and HdhQ 111 knockin transgenic mice.  ... 
<span class="external-identifiers"> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.1002/mds.25835">doi:10.1002/mds.25835</a> <a target="_blank" rel="external noopener" href="https://www.ncbi.nlm.nih.gov/pubmed/24573776">pmid:24573776</a> <a target="_blank" rel="external noopener" href="https://pubmed.ncbi.nlm.nih.gov/PMC4624289/">pmcid:PMC4624289</a> <a target="_blank" rel="external noopener" href="https://fatcat.wiki/release/lv3oofypczbcrpaiwsksn3y4ui">fatcat:lv3oofypczbcrpaiwsksn3y4ui</a> </span>
<a target="_blank" rel="noopener" href="https://web.archive.org/web/20200211083041/http://europepmc.org/backend/ptpmcrender.fcgi?accid=PMC4624289&amp;blobtype=pdf" title="fulltext PDF download" data-goatcounter-click="serp-fulltext" data-goatcounter-title="serp-fulltext"> <button class="ui simple right pointing dropdown compact black labeled icon button serp-button"> <i class="icon ia-icon"></i> Web Archive [PDF] <div class="menu fulltext-thumbnail"> <img src="https://blobs.fatcat.wiki/thumbnail/pdf/45/ce/45cef4211b5572a1c5ebae2108c08e75e06f4209.180px.jpg" alt="fulltext thumbnail" loading="lazy"> </div> </button> </a> <a target="_blank" rel="external noopener noreferrer" href="https://doi.org/10.1002/mds.25835"> <button class="ui left aligned compact blue labeled icon button serp-button"> <i class="external alternate icon"></i> wiley.com </button> </a> <a target="_blank" rel="external noopener" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4624289" title="pubmed link"> <button class="ui compact blue labeled icon button serp-button"> <i class="file alternate outline icon"></i> pubmed.gov </button> </a>
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