Assessing average somatic CAG repeat instability at the protein level.
Journal
Scientific reports
ISSN: 2045-2322
Titre abrégé: Sci Rep
Pays: England
ID NLM: 101563288
Informations de publication
Date de publication:
16 12 2019
16 12 2019
Historique:
received:
04
09
2019
accepted:
18
11
2019
entrez:
18
12
2019
pubmed:
18
12
2019
medline:
6
11
2020
Statut:
epublish
Résumé
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 MW1 Ab 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
Identifiants
pubmed: 31844074
doi: 10.1038/s41598-019-55202-x
pii: 10.1038/s41598-019-55202-x
pmc: PMC6915696
doi:
Substances chimiques
Antibodies
0
Huntingtin Protein
0
Peptides
0
polyglutamine
26700-71-0
DNA
9007-49-2
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
19152Références
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