eprintid: 1457419
rev_number: 35
eprint_status: archive
userid: 608
dir: disk0/01/45/74/19
datestamp: 2014-12-03 21:37:36
lastmod: 2021-10-04 00:49:51
status_changed: 2014-12-03 21:37:36
type: article
metadata_visibility: show
item_issues_count: 0
creators_name: Träger, U
creators_name: Andre, R
creators_name: Magnusson-Lind, A
creators_name: Miller, JR
creators_name: Connolly, C
creators_name: Weiss, A
creators_name: Grueninger, S
creators_name: Silajdžić, E
creators_name: Smith, DL
creators_name: Leavitt, BR
creators_name: Bates, GP
creators_name: Björkqvist, M
creators_name: Tabrizi, SJ
title: Characterisation of immune cell function in fragment and full-length Huntington's disease mouse models.
ispublished: pub
divisions: UCL
divisions: B02
divisions: C07
divisions: D07
divisions: F86
keywords: Animal models of disease, Cytokines, Huntington's disease, Innate immune system, Myeloid cells, Neurodegeneration, Neuroinflammation
note: © 2015 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license
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.
date: 2014-10-29
official_url: http://dx.doi.org/10.1016/j.nbd.2014.10.012
vfaculties: VFBRS
oa_status: green
full_text_type: pub
primo: open
primo_central: open_green
article_type_text: JOURNAL ARTICLE
verified: verified_manual
elements_source: PubMed
elements_id: 996385
doi: 10.1016/j.nbd.2014.10.012
pii: S0969-9961(14)00325-8
lyricists_name: Andre, Ralph
lyricists_name: Bates, Gillian
lyricists_name: Tabrizi, Sarah
lyricists_id: RANDR07
lyricists_id: GBATE91
lyricists_id: SJTAB21
full_text_status: public
publication: Neurobiol Dis
volume: 73C
pagerange: 388 - 398
citation:        Träger, U;    Andre, R;    Magnusson-Lind, A;    Miller, JR;    Connolly, C;    Weiss, A;    Grueninger, S;                         ... Tabrizi, SJ; + view all <#>        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, SJ;   - view fewer <#>    (2014)    Characterisation of immune cell function in fragment and full-length Huntington's disease mouse models.                   Neurobiol Dis , 73C    388 - 398.    10.1016/j.nbd.2014.10.012 <https://doi.org/10.1016/j.nbd.2014.10.012>.       Green open access   
 
document_url: https://discovery.ucl.ac.uk/id/eprint/1457419/1/1-s2.0-S0969996114003258-main.pdf