mHTT Seeding Activity: A Marker of Disease Progression and Neurotoxicity in Models of Huntington's Disease.

doi:10.1016/j.molcel.2018.07.032

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Article

mHTT Seeding Activity: A Marker of Disease Progression and Neurotoxicity in Models of Huntington's Disease.

Molecular cell - 6 Sept 2018

Ast Anne, Buntru Alexander, Schindler Franziska, Hasenkopf Regine, Schulz Aline, Brusendorf Lydia, Klockmeier Konrad, Grelle Gerlinde, McMahon Benjamin, Niederlechner Hannah, Jansen Isabelle, Diez Lisa, Edel Juliane, Boeddrich Annett, Franklin Sophie A, Baldo Barbara, Schnoegl Sigrid, Kunz Severine, Purfürst Bettina, Gaertner Annette, Kampinga Harm H, Morton A Jennifer, Petersén Åsa, Kirstein Janine, Bates Gillian P, Wanker Erich E

Abstract excerpt

Self-propagating, amyloidogenic mutant huntingtin (mHTT) aggregates may drive progression of Huntington's disease (HD). Here, we report the development of a FRET-based mHTT aggregate seeding (FRASE) assay that enables the quantification of mHTT seeding activity (HSA) in complex biosamples from HD patients and disease models. Application of the FRASE assay revealed HSA in brain homogenates of presymptomatic HD...

Topics

Animals
Animals, Genetically Modified
Biomarkers
Brain
Caenorhabditis elegans
Disease Models, Animal
Disease Progression
Drosophila

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Female

Humans

Huntingtin Protein

Huntington Disease

Male

Mice

Mice, Inbred C57BL

Mice, Inbred CBA

Mutation

Neurons

Source

Open in PubMed

Identifiers

PMID: 30193095
DOI: 10.1016/j.molcel.2018.07.032

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