Abstract：
:Inflammation is a growing area of research in neurodegeneration. In Huntington's disease (HD), a fatal inherited neurodegenerative disease caused by a CAG-repeat expansion in the gene encoding huntingtin, patients have increased plasma levels of inflammatory cytokines and circulating monocytes that are hyper-responsive to immune stimuli. Several mouse models of HD also show elevated plasma levels of inflammatory cytokines. To further determine the degree to which these models recapitulate observations in HD patients, we evaluated various myeloid cell populations from different HD mouse models to determine whether they are similarly hyper-responsive, as well as measuring other aspects of myeloid cell function. Myeloid cells from each of the three mouse models studied, R6/2, HdhQ150 knock-in and YAC128, showed increased cytokine production when stimulated. However, bone marrow CD11b(+) cells did not show the same hyper-responsive phenotype as spleen and blood cells. Furthermore, macrophages isolated from R6/2 mice show increased levels of phagocytosis, similar to findings in HD patients. Taken together, these results show significant promise for these mouse models to be used to study targeting innate immune pathways identified in human cells, thereby helping to understand the role the peripheral immune system plays in HD progression.
journal_name
Neurobiol Disjournal_title
Neurobiology of diseaseauthors
Träger U,Andre R,Magnusson-Lind A,Miller JR,Connolly C,Weiss A,Grueninger S,Silajdžić E,Smith DL,Leavitt BR,Bates GP,Björkqvist M,Tabrizi SJdoi
10.1016/j.nbd.2014.10.012subject
Has Abstractpub_date
2015-01-01 00:00:00pages
388-98eissn
0969-9961issn
1095-953Xpii
S0969-9961(14)00325-8journal_volume
73pub_type
杂志文章abstract：:Loss-of-function mutations in the parkin-encoding PARK2 gene are a frequent cause of young-onset, autosomal recessive Parkinson's disease (PD). Parkin knockout mice have no nigro-striatal neuronal loss but exhibit abnormalities of striatal dopamine transmission and cortico-striatal synaptic function. How these predege...
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journal_title：Neurobiology of disease
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journal_title：Neurobiology of disease
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abstract：UNLABELLED:Mitochondrial dysfunction is involved in the pathogenesis of motor neuron degeneration in the G93A mutant transgenic (tgmSOD1) animal model of ALS. However, it is unknown whether mitochondriopathy is a primary or secondary event. We isolated brain (BM) and spinal cord (SCM) mitochondria from 2 month old pres...
journal_title：Neurobiology of disease
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journal_title：Neurobiology of disease
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pub_type： 杂志文章,评审
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journal_title：Neurobiology of disease
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journal_title：Neurobiology of disease
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journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2017.07.014
更新日期：2017-10-01 00:00:00
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journal_title：Neurobiology of disease
pub_type： 杂志文章,评审
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journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2015.04.014
更新日期：2015-07-01 00:00:00
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journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2015.05.015
更新日期：2015-10-01 00:00:00
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journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2003.11.004
更新日期：2004-03-01 00:00:00
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journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2013.01.012
更新日期：2013-06-01 00:00:00
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journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2004.09.001
更新日期：2005-02-01 00:00:00
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journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2018.01.016
更新日期：2018-05-01 00:00:00
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journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2005.03.003
更新日期：2005-11-01 00:00:00
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journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2009.03.010
更新日期：2009-07-01 00:00:00
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journal_title：Neurobiology of disease
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journal_title：Neurobiology of disease
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doi：10.1016/j.nbd.2007.03.014
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pub_type： 杂志文章,评审
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journal_title：Neurobiology of disease
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journal_title：Neurobiology of disease
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journal_title：Neurobiology of disease
pub_type： 杂志文章,评审
doi：10.1016/j.nbd.2015.10.012
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abstract：:Seizures occur in the basal ganglia (BG) of epileptic patients and in animal models of epilepsy, but there is relatively little known about how these events are gated and/or propagated through this structure. Here, we present and characterize a model of in vitro seizure-like events (SLEs) in the striatum by applying c...
journal_title：Neurobiology of disease
pub_type： 杂志文章
doi：10.1016/j.nbd.2012.11.019
更新日期：2013-04-01 00:00:00
abstract：:The prevalence of central nervous system (CNS) neurologic dysfunction associated with human immunodeficiency virus (HIV) infection continues to increase, despite the use of antiretroviral therapy. Previous work has focused on the deleterious effects of HIV on mature neurons and on development of neuroprotective strate...
journal_title：Neurobiology of disease
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doi：10.1016/j.nbd.2010.12.002
更新日期：2011-03-01 00:00:00
