Journal
NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY
Volume 36, Issue 1, Pages 4-16Publisher
WILEY
DOI: 10.1111/j.1365-2990.2009.01045.x
Keywords
frontotemporal lobar degeneration; progranulin; tau; TDP-43; UBAP1
Categories
Funding
- Medical Research Council
- MRC [G0701441] Funding Source: UKRI
- Medical Research Council [G0701441] Funding Source: researchfish
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Frontotemporal lobar degeneration (FTLD) is a highly familial condition and is increasingly being recognized as an important form of dementia. The literature published on this disease is often difficult to collate due to the wide range in nomenclature used. Thankfully, consensus recommendations have now been published to address this issue and hopefully the community will adopt these as intended. Much progress has been made in our understanding of the clinical, pathological and genetic understanding of FTLD in recent years. Progranulin and TDP-43 have recently been identified as new important proteins involved in the pathophysiology of FTLD and this latter protein may have potential as a biomarker of this disease. However, much remains before we have a full picture of the genes that cause FTLD and the biological pathways in which they function. The purpose of this review is to summarize the current concepts and recent advances in our knowledge of this disease.
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