NEW YORK (Reuters Health) - Dutch and Belgian researchers have shown an inverse relationship between the length of an octapeptide repeat insertion in the prion protein gene and patients’ age at onset of dementia, according to a report in the August issue of the Journal of Neurology, Neurosurgery, and Psychiatry.
Dr. C. M. van Duijn of Erasmus Medical Center in Rotterdam, and colleagues, evaluated DNA from 17 patients with early-onset dementia. The investigators discovered a two-octapeptide repeat insertion in the prion protein gene in a patient who had developed dementia in his late fifties.
This prompted them to conduct a meta-analysis to investigate the relation of the number of repeats with age at onset of dementia and duration of illness.
The researchers were able to identify 24 articles describing prion protein octapeptide repeat insertions in 22 families. They identified 55 patients with the mutation. “We found an increasing number of repeats associated with younger age at onset.”
When they adjusted for age at onset, the investigators also found that duration of illness decreased significantly with the length of the repeat.
The authors point out that their findings help explain variations in age at onset of dementia. “The challenge in the future,” they conclude, “will be to determine the biological mechanism underlying this process.”
Source: J Neurol Neurosurg Psychiatry 2004; 75:1166-1170. [ Google search on this article ]
MeSH Headings:Biological Sciences: Biology: Genetics: Genetics, Medical: Health Occupations: Medicine: Age of Onset: Repetitive Sequences, Amino Acid: Biological SciencesCopyright © 2002 Reuters Limited. All rights reserved. Republication or redistribution of Reuters content, including by framing or similar means, is expressly prohibited without the prior written consent of Reuters. Reuters shall not be liable for any errors or delays in the content, or for any actions taken in reliance thereon. Reuters and the Reuters sphere logo are registered trademarks and trademarks of the Reuters group of companies around the world.
