Permanent URL to this publication: http://dx.doi.org/10.5167/uzh-27482
Neumann, M; Tolnay, M; Mackenzie, I R A (2009). The molecular basis of frontotemporal dementia. Expert Reviews in Molecular Medicine, 11:e23.
Frontotemporal dementia (FTD) is a clinical syndrome with a heterogeneous molecular basis. Familial FTD has been linked to mutations in several genes, including those encoding the microtubule-associated protein tau (MAPT), progranulin (GRN), valosin-containing protein (VCP) and charged multivescicular body protein 2B (CHMP2B). The associated neuropathology is characterised by selective degeneration of the frontal and temporal lobes (frontotemporal lobar degeneration, FTLD), usually with the presence of abnormal intracellular protein accumulations. The current classification of FTLD neuropathology is based on the identity of the predominant protein abnormality, in the belief that this most closely reflects the underlying pathogenic process. Major subgroups include those characterised by the pathological tau, TDP-43, intermediate filaments and a group with cellular inclusions composed of an unidentified ubiquitinated protein. This review will focus on the current understanding of the molecular basis of each of the major FTLD subtypes. It is anticipated that this knowledge will provide the basis of future advances in the diagnosis and treatment of FTD.
|Item Type:||Journal Article, refereed, further contribution|
|Communities & Collections:||04 Faculty of Medicine > University Hospital Zurich > Institute of Neuropathology|
|DDC:||570 Life sciences; biology
610 Medicine & health
|Date:||29 July 2009|
|Deposited On:||30 Jan 2010 16:58|
|Last Modified:||27 Nov 2013 19:42|
|Publisher:||Cambridge University Press|
|Additional Information:||Copyright: Cambridge University Press|
|Citations:||Web of Science®. Times Cited: 33|
Scopus®. Citation Count: 39
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