A5068287616- Genetic Neurodegenerative Diseases
- Mitochondrial Function and Pathology
- Muscle Physiology and Disorders
- Muscle metabolism and nutrition
- Neurological disorders and treatments
- Neuroscience and Neuropharmacology Research
University of Groningen
2023-2025
A family of neurodegenerative diseases, including Huntington's disease (HD) and spinocerebellar ataxias, are associated with an abnormal polyglutamine (polyQ) expansion in mutant proteins that become prone to form amyloid-like aggregates. Prior studies have suggested a key role for β-hairpin formation as driver nucleation aggregation, but direct experimental been challenging. Toward such research, we set out enable spatiotemporal control over by the introduction photosensitive β-turn mimic...
Abstract Huntington’s disease (HD) is a neurodegenerative disorder in which mutated fragments of the huntingtin protein (Htt) undergo misfolding and aggregation. Since aggregated proteins can cause cellular stress cytotoxicity, there an interest development small molecule aggregation inhibitors as potential modulators HD pathogenesis. Here, we study how polyphenol modulates mechanism exon 1 (HttEx1) even at sub-stoichiometric ratios. Sub-stoichiometric amounts curcumin impacted primary...
Antibodies are critical for the immune response and serve as important tools due to their ability recognize specific amino acid sequences, or epitopes. Based on latter, they utilized diagnostic in biological biomedical research. Huntington's disease (HD) is a neurodegenerative condition caused by CAG repeat expansions huntingtin (HTT) gene characterized amyloid-like protein deposits patients. Multiple anti-HTT antibodies used HD research HTT inclusions both post-mortem tissue laboratory...
Neurodegeneration in Huntington's disease (HD) is accompanied by the aggregation of fragments mutant huntingtin protein, a biomarker progression. A particular pathogenic role has been attributed to aggregation-prone exon 1 (HTTex1), generated aberrant splicing or proteolysis, and containing expanded polyglutamine (polyQ) segment. Unlike amyloid fibrils from Parkinson's Alzheimer's diseases, atomic-level structure HTTex1 remained unknown, limiting diagnostic treatment efforts. We present...
Neurodegeneration in Huntington's disease (HD) is accompanied by the aggregation of fragments mutant huntingtin protein, a biomarker progression. A particular pathogenic role has been attributed to aggregation-prone exon 1 (HTTex1), generated aberrant splicing or proteolysis, and containing expanded polyglutamine (polyQ) segment. Unlike amyloid fibrils from Parkinson's Alzheimer's diseases, atomic-level structure HTTex1 remained unknown, limiting diagnostic treatment efforts. We present...
Abstract Huntington’s disease (HD) is a neurodegenerative disorder in which mutated fragments of the huntingtin protein (Htt) undergo misfolding and aggregation. Since misfolded aggregated proteins can cause cellular stress cytotoxicity, there an interest development small molecule aggregation inhibitors as potential modulators HD pathogenesis. Here, we study how polyphenol modulates mechanism exon 1 (HttEx1) even at sub-stoichiometric ratios. Sub-stoichiometric amounts curcumin impacted...