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Updated: Sep 29, 2026

Measuring RAN Peptide Toxicity in C. elegans
Published on: April 30, 2020
Properties of neurotoxic peptides related to the BRI gene
B Austen1, O el-Agnaf, S Nagala
1Neurodegeneration Unit, Surgery Department, St. George's Hospital Medical School, Cranmer Terrace, London SW17 ORE, UK. sghk200@sghms.a.cuk
Abstract:
Mutations in the BRI gene are thought to cause dementias in members of families. The clinical symptoms are similar to those of Alzheimer's disease, but with additional ocular and hearing deficits, and spasticity. The mutations lead to the release of the 34-residue peptides, ABri and ADan, in the brains of afflicted individuals. We have synthesized the peptides in their straight-chain and oxidized cyclic forms and shown that the oxidized form of ABri and reduced form of ADan are toxic to human neuronal cell lines in culture. Neurotoxicity correlates with the extent of formation of SDS-stable non-fibrillar low-molecular-mass oligomers (SSNFOs).
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