Assessing average somatic CAG repeat instability at the protein level
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F67985904%3A_____%2F19%3A00518791" target="_blank" >RIV/67985904:_____/19:00518791 - isvavai.cz</a>
Result on the web
<a href="https://www.nature.com/articles/s41598-019-55202-x" target="_blank" >https://www.nature.com/articles/s41598-019-55202-x</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1038/s41598-019-55202-x" target="_blank" >10.1038/s41598-019-55202-x</a>
Alternative languages
Result language
angličtina
Original language name
Assessing average somatic CAG repeat instability at the protein level
Original language description
Sandwich ELISA-based methods use Abs that target the expanded polyglutamine (polyQ) tract to quantify mutant huntingtin (mHTT). Using Meso Scale Discovery (MSD) assay, the mHTT signal detected with MW1Ab correlated with polyQ length and doubled with a difference of only 7 glutamine residues between equivalent amounts of purified mHTTexon1 proteins. Similar polyQ length-dependent effects on MSD signals were confirmed using endogenous full length mHTT from brains of Huntington's disease (HD) knock-in (KI) mice. We used this avidity bias to devise a method to assess average CAG repeat instability at the protein level in a mixed population of HTT proteins present in tissues. Signal detected for average polyQ length quantification at the protein level by our method exhibited a strong correlation with average CAG repeat length at the genomic DNA level determined by PCR method in striatal tissue homogenates from Hdh(Q140) KI mice and in human HD postmortem cortex. This work establishes that CAG repeat instability in mutant HTT is reflected at the protein level.
Czech name
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Czech description
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Classification
Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
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OECD FORD branch
30402 - Technologies involving the manipulation of cells, tissues, organs or the whole organism (assisted reproduction)
Result continuities
Project
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Continuities
I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace
Others
Publication year
2019
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
Scientific Reports
ISSN
2045-2322
e-ISSN
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Volume of the periodical
9
Issue of the periodical within the volume
DEC 16
Country of publishing house
GB - UNITED KINGDOM
Number of pages
14
Pages from-to
19152
UT code for WoS article
000503171600001
EID of the result in the Scopus database
2-s2.0-85076559713