Počet záznamů: 1
Assessing average somatic CAG repeat instability at the protein level
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SYSNO ASEP 0518791 Druh ASEP J - Článek v odborném periodiku Zařazení RIV J - Článek v odborném periodiku Poddruh J Článek ve WOS Název Assessing average somatic CAG repeat instability at the protein level Tvůrce(i) Aviolat, H. (US)
Mouro Pinto, R. (US)
Godshall, E. (US)
Murtha, R. (US)
Richey, H. E. (US)
Sapp, E. (US)
Vodička, Petr (UZFG-Y) ORCID
Wheeler, W. C. (US)
Kegel-Gleason, K. B. (US)
DiFiglia, M. (US)Číslo článku 19152 Zdroj.dok. Scientific Reports. - : Nature Publishing Group - ISSN 2045-2322
Roč. 9, DEC 16 (2019)Poč.str. 14 s. Forma vydání Online - E Jazyk dok. eng - angličtina Země vyd. GB - Velká Británie Klíč. slova mutant huntingtin ; CAG Vědní obor RIV EI - Biotechnologie a bionika Obor OECD Technologies involving the manipulation of cells, tissues, organs or the whole organism (assisted reproduction) Způsob publikování Open access Institucionální podpora UZFG-Y - RVO:67985904 UT WOS 000503171600001 EID SCOPUS 85076559713 DOI 10.1038/s41598-019-55202-x Anotace 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. Pracoviště Ústav živočišné fyziologie a genetiky Kontakt Jana Zásmětová, knihovna@iapg.cas.cz, Tel.: 315 639 554 Rok sběru 2020 Elektronická adresa https://www.nature.com/articles/s41598-019-55202-x
Počet záznamů: 1