Frontotemporal Dementias
4th International Conference
Lund, Sweden, April 24-26, 2003
Editors: Arne Brun, Lund; Elisabet Englund, Lund; Lars Gustafson, Lund; Ulla Passant, Lund; Jarl Risberg, Lund

Paper

The Tau Gene Locus and Frontotemporal Dementia
Stuart Pickering-Brown

Institute of Psychiatry, London, UK

Address of Corresponding Author

Dement Geriatr Cogn Disord 2004;17:258-260 (DOI: 10.1159/000077149)

  Key Words

• Frontotemporal dementia
• Tau FTDP-17
• Neuronal inclusions

  Abstract

Frontotemporal lobar degeneration (FTLD) has become an increasingly recognised form of dementia. It has been demonstrated that a proportion of cases of FTLD result from mutations in the tau gene on chromosome 17. A pathological hallmark in the brains from patients with tau mutations is an accumulation of insoluble tau within neurons and glia that is considered neurotoxic. However, there are reports of families with FTLD linked to the tau locus on chromosome 17 in which no mutations have been found, and these families lack any accumulation of insoluble tau. Recently, two new tau mutations have been described that also appear to cause disease in the absence of any tau accumulation. The role of the tau locus and tau accumulation in contributing to the neurodegenerative process in chromosome-17-linked families without mutations and in families with tau mutations without insoluble tau is discussed.

Copyright © 2004 S. Karger AG, Basel

  Author Contacts

S. Pickering-Brown, PhD
Institute of Psychiatry, Section of Old Age Psychiatry
Denmark Hill, London, SE5 8AF (UK)
Tel. +44 207 848 0346, Fax +44 207 848 0632
E-Mail S.Pickering-Brown@iop.kcl.ac.uk

  Article Information

Number of Print Pages : 3
Number of Figures : 0, Number of Tables : 0, Number of References : 31