Properties of neurotoxic peptides related to the BRI gene

被引：8

作者：

Austen, B ^{[1
]}

El-Agnaf, O ^{[1
]}

Nagala, S ^{[1
]}

Patel, B ^{[1
]}

Gunasekera, N ^{[1
]}

Lee, M ^{[1
]}

Lelyveld, V ^{[1
]}

机构：

[1] St George Hosp, Sch Med, Dept Surg, Neurodegenerat Unit, London SW17 0RE, England

来源：

BIOCHEMICAL SOCIETY TRANSACTIONS | 2002年 / 30卷

关键词：

British amyloid; Danish amyloid; dementia; neurotoxicity;

D O I：

10.1042/bst0300557

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 [生物化学与分子生物学]; 081704 [应用化学];

摘要：

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 culture. Neurotoxicity correlates with the extent of formation of SDS-stable non-fibrillar low-molecular-mass oligomers (SSNFOs).

引用

页码：557 / 559

页数：3

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