HOME >> BIOLOGY >> NEWS
Gene that causes familial British dementia may yield clues to Alzheimer's disease

Researchers supported by the National Institute on Aging (NIA) have discovered a novel gene, which when mutated is responsible for familial British dementia (FBD), a rare inherited disease that causes progressive dementia like that seen in patients with Alzheimer's disease (AD) and severe movement disorders. This finding provides an exciting new clue to abnormal changes in the brain that lead to dementia.

FBD, previously called familial cerebral amyloid angiopathy, was first reported in the 1940s. This autosomal dominant disorder has since been described in a large British family of more than 300 members spanning 9 generations. The most common symptoms of this devastating disease -- which usually develops in patients in their 40s and 50s -- are dementia and spasticity. A distinct feature of FBD is the development of Alzheimer's disease-like dementia instead of stroke, which is common in other inherited forms of cerebral angiopathy.

Researchers at the New York University School of Medicine in collaboration with scientists at the National Hospital for Neurology and Neurosurgery and the Institute of Neurology, both in London, England, discovered a novel gene, BRI, located on chromo-some 13. A point mutation at the stop codon of this gene results in the production of a longer-than-normal BRI precursor protein. A peptide (ABri peptide) snipped from the mutated end of the BRI precursor protein is deposited as amyloid fibrils, which are thought to contribute to neuronal dysfunction and dementia. The research is reported in the June 24, 1999, issue of Nature (Vidal, R; Frangione, B; Rostagno, A; Mead, S; Revesz, T; Plant, G and Ghiso, J "A stop-codon mutation in the BRI gene associated with familial British dementia," Nature June 24, 1999).

The research group identified the mutated gene in a female FBD family member, who developed the disease at age 56 and died at age 65. They also found the mutation in 7 affected FBD family members, but not in unaff
'"/>

Contact: Karen Poncinki/Stephanie Clipper
clippers@exmur.nia.nih.gov
301-496-1752
NIH/National Institute on Aging
23-Jun-1999


Page: 1 2 3

Related biology news :

1. Cigarette smoke causes breaks in DNA and defects to a cells chromosomes, Pitt study finds
2. Pollutant causes delayed flowering in plants
3. Disruption of protein-folding causes neurodegeneration, mental retardation
4. Researchers discover why mutant gene causes colon cancer
5. Analysis fingers causes of desertification
6. Vioxx in high doses causes hypertension
7. Gene mutation causes progressive changes to cell structure in children with Progeria
8. Late invasion of infected cells into the brain causes HIV dementia say Temple researchers
9. Fat cell hormone causes weight loss
10. Lack of specific brain protein causes marked deficits in learning, memory
11. UIC researchers discover gene that causes liver cancer in animals

Post Your Comments:
(Date:10/29/2014)... stem cell, those referred to as ,facultative, form part—together ... nothing that differentiates these cells from the others. However, ... the capacity to become stem cells again. This phenomenon ... that hosts cells that stimulate tissue growth, thus allowing ... a transplant. Knowledge of the underlying mechanism that ...
(Date:10/29/2014)... Ghrelin is a hormone released by the stomach and ... viewed as a psychoactive substance that primarily affects brain ... , This knowledge, combined with findings from animal ... the potential to stimulate alcohol craving. , Dr. Lorenzo ... found that, as they had anticipated, alcohol craving was ...
(Date:10/28/2014)... . , How ... when times are lean? By studying wild chimpanzees in ... the Max Planck Institute for Evolutionary Anthropology in Leipzig, ... can acquire extra energy needed to maintain large, costly ... more en route to breakfast sites containing fruits that ...
Breaking Biology News(10 mins):A mechanism that allows a differentiated cell to reactivate as a stem cell revealed 2Ghrelin stimulates an appetite for drinking alcohol 2The early chimp gets the fig 2The early chimp gets the fig 3
(Date:10/30/2014)... Al Khaimah, UAE (PRWEB) October 30, 2014 ... appointment of Dr. James A. Manganello, Chairman of the Board, ... of The Bahamas. The appointment was made by the ... Bahamas on September 24th, 2014. , More than ... Therapy and Research Bill , Prime Minister Perry Christie said ...
(Date:10/30/2014)... of the key challenges in the development of quantum ... , In a paper published today (28 October) in ... show how to make a new type of flexibly ... such as high-precision sensors and specialised superfast computers, often ... the methods for trapping these tiny particles are hugely ...
(Date:10/27/2014)... 2014 The Italian company ... and sell its artificial "lower limb", after the ...      (Photo: http://photos.prnewswire.com/prnh/20141027/713097 ) ... the definitive judgement has arrived: Roadrunnerfoot, the small ... accessible hi-tech prostheses with composite materials, has won ...
(Date:10/27/2014)... (PRWEB) October 27, 2014 New ... SoundConnect , an industry leading unified communication ... partners and agents to deliver cloud-based audio and ... growth opportunities. , With SoundConnect’s Q4 incentive ... twenty-five video and/or web conferencing licenses sold, now ...
Breaking Biology Technology:Grace Century’s Stem Cell Biobank Project Announces Chairman’s Appointment to National Stem Cell Ethics Committee of The Bahamas 2Grace Century’s Stem Cell Biobank Project Announces Chairman’s Appointment to National Stem Cell Ethics Committee of The Bahamas 3Italian Lower Limb Prosthesis Company Wins Lawsuit Against German Giant Ottobock 2SoundConnect Unveils Q4 Partner Incentives 2
Cached News: