The genetics of frontotemporal lobar degeneration.
OBJECTIVE
This review addresses the latest developments in the genetics of frontotemporal lobar degeneration. 'Frontotemporal lobar degeneration' is the clinical term used to describe a heterogeneous neurodegenerative syndrome that includes frontotemporal dementia, semantic dementia, progressive nonfluent aphasia and progressive apraxia. Up to 40% of patients with frontotemporal lobar degeneration have a family history of a similar disorder in a first-degree relative, highlighting a significant genetic contribution to the aetiology of this disorder.


RESULTS
Four genes that cause autosomal frontotemporal lobar degeneration have already been identified, including two that are only 1.7 megabases apart on chromosome 17.


CONCLUSIONS
Although much progress has been made in our understanding of the genetics of frontotemporal lobar degeneration in recent years, the majority of the genetic causes of this syndrome remains to be identified.
Sikkink
Stephen
S
Clinical Neurosciences, University of Manchester, Manchester, UK.
Rollinson
Sara
S
Pickering-Brown
Stuart M
SM
D001921:Brain / Q000378:metabolism* / Q000503:physiopathology; D002874:Chromosome Mapping; D002875:Chromosomes / Q000235:genetics; D004252:DNA Mutational Analysis; D003704:Dementia / Q000235:genetics* / Q000378:metabolism / Q000503:physiopathology; D020022:Genetic Predisposition to Disease / Q000235:genetics*; D006801:Humans; D009154:Mutation / Q000235:genetics*; D009419:Nerve Tissue Proteins / Q000235:genetics; D012307:Risk Factors
D009419:Nerve Tissue Proteins