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:
*Name:
*Comment:
*Email:


(Date:11/30/2016)... FRANCISCO and WARSAW, Poland , Nov. 30, 2016 /PRNewswire/ ... It is one of the most crucial aspects of recovery so we need to ... of serious health risks, including heart problems, high blood pressure, stroke, diabetes, and even ... and friends sleep and find a Christmas present that could help them to manage ... ...
(Date:11/29/2016)... Nov. 29, 2016   Neurotechnology , ... object recognition technologies, today released FingerCell 3.0, ... recognition solutions that run on low-power, low-memory ... using less than 128KB of memory, enabling ... that have limited on-board resources, such as: ...
(Date:11/24/2016)... -- Cercacor today introduced Ember TM Sport Premium ... measure hemoglobin, Oxygen Content, Oxygen Saturation, Perfusion Index, ... approximately 30 seconds. Smaller than a smartphone, using only ... key data about their bodies to help monitor these ... Hemoglobin carries oxygen to muscles. When hemoglobin and ...
Breaking Biology News(10 mins):
(Date:12/8/2016)... SAN DIEGO , Dec. 8, 2016 /PRNewswire-USNewswire/ ... treatments for congestive heart failure and type 2 ... license for a novel adeno-associated virus (AAV) vector ... Kay , M.D., Ph.D., at Stanford University. The ... of its paracrine gene therapy product pipeline. ...
(Date:12/8/2016)... 8, 2016 Oxford Gene Technology ... SureSeqâ„¢ NGS panel range with the launch of the SureSeq ... study of variants in familial hypercholesterolemia (FH). The panel delivers ... on a single small panel and allows customisation by ,mix ... all exons for LDLR , P C ...
(Date:12/8/2016)... (PRWEB) , ... December 08, 2016 , ... This CAST ... approvals for biotech crops. The authors focus on the economic effects in countries that ... approval of new biotech crops and the resultant risk of low level presence (LLP) ...
(Date:12/8/2016)... SEOUL, South Korea , Dec. 8, 2016 /PRNewswire/ ... completed a $21 billion KRW (US $18.9M) Series A ... Management, Kolon Investment, G.N. Tech Venture and SNU Bio ... by Eutilex to 30.5 billion KRW (US $27.7M) since ... will help Eutilex to bolster the development and commercialization ...
Breaking Biology Technology:
Cached News: