Properties of neurotoxic peptides related to the BRI gene

B. Austen, O. El-Agnaf, S. Nagala, B. Patel, N. Gunasekera, M. Lee, V. Lelyveld

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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).

Original languageEnglish
Pages (from-to)557-559
Number of pages3
JournalBiochemical Society Transactions
Volume30
Issue number4
DOIs
Publication statusPublished - 1 Aug 2002

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Keywords

  • British amyloid
  • Danish amyloid
  • Dementia
  • Neurotoxicity

ASJC Scopus subject areas

  • Biochemistry

Cite this

Austen, B., El-Agnaf, O., Nagala, S., Patel, B., Gunasekera, N., Lee, M., & Lelyveld, V. (2002). Properties of neurotoxic peptides related to the BRI gene. Biochemical Society Transactions, 30(4), 557-559. https://doi.org/10.1042/BST0300557