Structure and neurotoxicity of novel amyloids derived from the BRI gene

G. Gibson, Omar Ali El-Agnaf, Z. Anwar, C. Sidera, A. Isbister, B. M. Austen

Research output: Contribution to journalArticle

8 Citations (Scopus)

Abstract

A number of human neurodegenerative diseases involve aggregated amyloid proteins in the brain, e.g. Alzheimer's disease (β-amyloid) and Parkinson's disease (α-synuclein). Other examples are rare familial dementias which involve the BRI gene. In a British family, mutation of the termination codon extends the reading frame of BRI to yield a furin-processed 34-residue peptide (Abri; British dementia peptide), 11 residues longer than the wild-type (WT). In a Danish family, a ten-base insertion also yields a 34-residue peptide (Adan; Danish dementia peptide). To explore the roles of Abri and Adan in neurodegeneration, we synthesized Abri and Adan in oxidized and reduced forms and generated transgenic mice colonies expressing the WT and mutated forms of BRI. We have generated transgenic mice colonies bearing the genes coding for WT-BRI, Adan and Abri under the control of the Thy1 promoter. Whereas WT-BRI transgenic mice express full-length WT-BRI protein in their brains, Adan protein is fully processed to small peptides.

Original languageEnglish
Pages (from-to)1111-1112
Number of pages2
JournalBiochemical Society Transactions
Volume33
Issue number5
DOIs
Publication statusPublished - Nov 2005
Externally publishedYes

Fingerprint

Abrus
Amyloid
Genes
Peptides
Transgenic Mice
Dementia
Brain
Bearings (structural)
Synucleins
Furin
Neurodegenerative diseases
Amyloidogenic Proteins
Reading Frames
Terminator Codon
Neurodegenerative Diseases
Parkinson Disease
Alzheimer Disease
Proteins
Mutation

Keywords

  • α-synuclein
  • β-amyloid
  • BRI gene
  • Danish dementia peptide (Adan)
  • Neurotoxicity
  • Transgenic mice colony

ASJC Scopus subject areas

  • Biochemistry

Cite this

Structure and neurotoxicity of novel amyloids derived from the BRI gene. / Gibson, G.; Ali El-Agnaf, Omar; Anwar, Z.; Sidera, C.; Isbister, A.; Austen, B. M.

In: Biochemical Society Transactions, Vol. 33, No. 5, 11.2005, p. 1111-1112.

Research output: Contribution to journalArticle

Gibson, G. ; Ali El-Agnaf, Omar ; Anwar, Z. ; Sidera, C. ; Isbister, A. ; Austen, B. M. / Structure and neurotoxicity of novel amyloids derived from the BRI gene. In: Biochemical Society Transactions. 2005 ; Vol. 33, No. 5. pp. 1111-1112.
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