Properties of neurotoxic peptides related to the BRI gene

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

doi:10.1042/BST0300557

Properties of neurotoxic peptides related to the BRI gene

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

^*Corresponding author for this work

St. George's University of London

Research output: Contribution to journal › Article › peer-review

9 Citations (Scopus)

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 language	English
Pages (from-to)	557-559
Number of pages	3
Journal	Biochemical Society Transactions
Volume	30
Issue number	4
DOIs	https://doi.org/10.1042/BST0300557
Publication status	Published - Aug 2002
Externally published	Yes

Keywords

British amyloid
Danish amyloid
Dementia
Neurotoxicity

Access to Document

10.1042/BST0300557

Cite this

@article{8dd39f738c804799bf9a243e77eb006b,

title = "Properties of neurotoxic peptides related to the BRI gene",

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).",

keywords = "British amyloid, Danish amyloid, Dementia, Neurotoxicity",

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

year = "2002",

month = aug,

doi = "10.1042/BST0300557",

language = "English",

volume = "30",

pages = "557--559",

journal = "Biochemical Society Transactions",

issn = "0300-5127",

publisher = "Portland Press Ltd",

number = "4",

}

TY - JOUR

T1 - Properties of neurotoxic peptides related to the BRI gene

AU - Austen, B.

AU - El-Agnaf, O.

AU - Nagala, S.

AU - Patel, B.

AU - Gunasekera, N.

AU - Lee, M.

AU - Lelyveld, V.

PY - 2002/8

Y1 - 2002/8

N2 - 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).

AB - 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).

KW - British amyloid

KW - Danish amyloid

KW - Dementia

KW - Neurotoxicity

UR - http://www.scopus.com/inward/record.url?scp=0036671447&partnerID=8YFLogxK

U2 - 10.1042/BST0300557

DO - 10.1042/BST0300557

M3 - Article

C2 - 12196136

AN - SCOPUS:0036671447

SN - 0300-5127

VL - 30

SP - 557

EP - 559

JO - Biochemical Society Transactions

JF - Biochemical Society Transactions

IS - 4

ER -

Properties of neurotoxic peptides related to the BRI gene

Abstract

Keywords

Access to Document

Other files and links

Fingerprint

Cite this