A transgenic minipig model of Hungtington´s disease
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F67985556%3A_____%2F13%3A00391811" target="_blank" >RIV/67985556:_____/13:00391811 - isvavai.cz</a>
Alternative codes found
RIV/67985904:_____/13:00391811
Result on the web
<a href="http://dx.doi.org/10.3233/JHD-130001" target="_blank" >http://dx.doi.org/10.3233/JHD-130001</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.3233/JHD-130001" target="_blank" >10.3233/JHD-130001</a>
Alternative languages
Result language
angličtina
Original language name
A transgenic minipig model of Hungtington´s disease
Original language description
Background: 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. Methods: 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 HTT mRNA and protein fragment were detected in brain and peripheral tissues. No aggregate formation in brain up to 16 months was seen by AGERA and filter
Czech name
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Czech description
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Classification
Type
J<sub>x</sub> - Unclassified - Peer-reviewed scientific article (Jimp, Jsc and Jost)
CEP classification
EB - Genetics and molecular biology
OECD FORD branch
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Result continuities
Project
Result was created during the realization of more than one project. More information in the Projects tab.
Continuities
I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace
Others
Publication year
2013
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
Journal of Huntington´s Disease
ISSN
1879-6397
e-ISSN
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Volume of the periodical
2
Issue of the periodical within the volume
1
Country of publishing house
NL - THE KINGDOM OF THE NETHERLANDS
Number of pages
22
Pages from-to
47-68
UT code for WoS article
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EID of the result in the Scopus database
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