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Statements

Subject Item
n2:RIV%2F00027162%3A_____%2F13%3A%230001011%21RIV14-MZE-00027162
rdf:type
skos:Concept n17:Vysledek
dcterms:description
Some promising treatments for Huntington's disease (HD) may require pre-clinical testing in large animals. Minipig is a suitable species because of its large gyrencephalic brain and long lifespan. Objective: To generate HD transgenic (TgHD) minipigs encoding huntingtin (HTT)1-548 under the control of human HTT promoter. Transgenesis was achieved by lentiviral infection of porcine embryos. PCR assessment of gene transfer, observations of behavior, and postmortem biochemical and immunohistochemical studies were conducted. Results: One copy of the human HTT transgene encoding 124 glutamines integrated into chromosome 1 q24-q25 and successful germ line transmission occurred through successive generations (F0, F1, F2 and F3 generations). No developmental or gross motor deficits were noted up to 40 months of age. Mutant HTTmRNAand protein fragment were detected in brain and peripheral tissues. No aggregate formation in brain up to 16 months was seen by AGERA and filter retardation or by immunostaining. DARPP32 labeling in WT and TgHD minipig neostriatum was patchy. Analysis of 16 month old siblings showed reduced intensity of DARPP32 immunoreactivity in neostriatal TgHD neurons compared to those of WT. Compared to WT, TgHD boars by one year had reduced fertility and fewer spermatozoa per ejaculate. In vitro analysis revealed a significant decline in the number of WT minipig oocytes penetrated by TgHD spermatozoa. The findings demonstrate successful establishment of a transgenic model of HD in minipig that should be valuable for testing long term safety of HD therapeutics. The emergence of HD-like phenotypes in the TgHD minipigs will require more study Some promising treatments for Huntington's disease (HD) may require pre-clinical testing in large animals. Minipig is a suitable species because of its large gyrencephalic brain and long lifespan. Objective: To generate HD transgenic (TgHD) minipigs encoding huntingtin (HTT)1-548 under the control of human HTT promoter. Transgenesis was achieved by lentiviral infection of porcine embryos. PCR assessment of gene transfer, observations of behavior, and postmortem biochemical and immunohistochemical studies were conducted. Results: One copy of the human HTT transgene encoding 124 glutamines integrated into chromosome 1 q24-q25 and successful germ line transmission occurred through successive generations (F0, F1, F2 and F3 generations). No developmental or gross motor deficits were noted up to 40 months of age. Mutant HTTmRNAand protein fragment were detected in brain and peripheral tissues. No aggregate formation in brain up to 16 months was seen by AGERA and filter retardation or by immunostaining. DARPP32 labeling in WT and TgHD minipig neostriatum was patchy. Analysis of 16 month old siblings showed reduced intensity of DARPP32 immunoreactivity in neostriatal TgHD neurons compared to those of WT. Compared to WT, TgHD boars by one year had reduced fertility and fewer spermatozoa per ejaculate. In vitro analysis revealed a significant decline in the number of WT minipig oocytes penetrated by TgHD spermatozoa. The findings demonstrate successful establishment of a transgenic model of HD in minipig that should be valuable for testing long term safety of HD therapeutics. The emergence of HD-like phenotypes in the TgHD minipigs will require more study
dcterms:title
A Transgenic Minipig Model of Huntingtons Disease A Transgenic Minipig Model of Huntingtons Disease
skos:prefLabel
A Transgenic Minipig Model of Huntingtons Disease A Transgenic Minipig Model of Huntingtons Disease
skos:notation
RIV/00027162:_____/13:#0001011!RIV14-MZE-00027162
n17:predkladatel
n18:ico%3A00027162
n3:aktivita
n6:Z n6:I n6:S
n3:aktivity
I, S, Z(MZE0002716202)
n3:cisloPeriodika
1
n3:dodaniDat
n10:2014
n3:domaciTvurceVysledku
n15:3191907 n15:8408033
n3:druhVysledku
n19:J
n3:duvernostUdaju
n4:S
n3:entitaPredkladatele
n13:predkladatel
n3:idSjednocenehoVysledku
59165
n3:idVysledku
RIV/00027162:_____/13:#0001011
n3:jazykVysledku
n12:eng
n3:klicovaSlova
Huntington's disease; mutant huntingtin; minipigs; large animal model; lentiviral transgenesis; FISH analysis; mRNA; and protein expression; immunohistochemistry; DARPP32; AGERA assay; TR-FRET assay; spermatozoa
n3:klicoveSlovo
n7:TR-FRET%20assay n7:AGERA%20assay n7:minipigs n7:mutant%20huntingtin n7:and%20protein%20expression n7:DARPP32 n7:spermatozoa n7:immunohistochemistry n7:Huntington%27s%20disease n7:FISH%20analysis n7:large%20animal%20model n7:mRNA n7:lentiviral%20transgenesis
n3:kodStatuVydavatele
NL - Nizozemsko
n3:kontrolniKodProRIV
[1C3F03FD5D2F]
n3:nazevZdroje
Journal of Huntington's Disease
n3:obor
n16:EB
n3:pocetDomacichTvurcuVysledku
2
n3:pocetTvurcuVysledku
24
n3:rokUplatneniVysledku
n10:2013
n3:svazekPeriodika
2
n3:tvurceVysledku
Musilová, Petra Kubíčková, Svatava
n3:zamer
n9:MZE0002716202
s:issn
1879-6397
s:numberOfPages
22
n14:doi
10.3233/JHD-130001