Meso scale discovery-based assays for the detection of aggregated huntingtin

• Published in: PloS one Vol. 14; no. 3; p. e0213521
• Main Authors: Reindl, Wolfgang, Baldo, Barbara, Schulz, Jana, Janack, Isabell, Lindner, Ilka, Kleinschmidt, Markus, Sedaghat, Yalda, Thiede, Christina, Tillack, Karsten, Schmidt, Christina, Cardaun, Isabell, Schwagarus, Tom, Herrmann, Frank, Hotze, Madlen, Osborne, Georgina F, Herrmann, Simone, Weiss, Andreas, Zerbinatti, Celina, Bates, Gillian P, Bard, Jonathan, Munoz-Sanjuan, Ignacio, Macdonald, Douglas
• Format: Journal Article
• Language: English
• Published: United States Public Library of Science 26.03.2019; Public Library of Science (PLoS)
• Subjects: Age; Alzheimer's disease; Alzheimers disease; Amino acids; Animal models; Animals; Assaying; Biological Assay; Biological properties; Biological samples; Biology and Life Sciences; Brain; Brain - metabolism; Change detection; Chromatography; Clinical trials; Cortex; Dementia; Diagnosis; Discovery and exploration; Disease Models, Animal; Drug development; Economic models; Enzyme-linked immunosorbent assay; Genes; Glutamine; Humans; Huntingtin; Huntingtin Protein - genetics; Huntingtin Protein - metabolism; Huntington Disease - genetics; Huntington Disease - metabolism; Huntington Disease - pathology; Huntington's disease; Huntingtons disease; Intracellular; Lysates; Medical research; Medicine and Health Sciences; Mice; Mice, Transgenic; Monomers; Neostriatum; Nervous system diseases; Neurodegenerative diseases; Neuropathology; Peptides - genetics; Peptides - metabolism; Physical Sciences; Polyglutamine; Protein Aggregation, Pathological - genetics; Protein Aggregation, Pathological - metabolism; Protein Aggregation, Pathological - pathology; Proteins; Research and Analysis Methods; Size exclusion chromatography; Species; Supervision; Technology; Therapeutics; Time dependence; Trinucleotide repeats; University colleges; Zinc; Zinc finger proteins; Los Angeles California; United Kingdom > UK; United States > US; Germany

Huntington's disease (HD) is a monogenic neurodegenerative disorder caused by an expansion of the CAG trinucleotide repeat domain in the huntingtin (HTT) gene, leading to an expanded poly-glutamine (polyQ) stretch in the HTT protein. This mutant HTT (mHTT) protein is highly prone to intracellul...