Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:

 

Researchers surprised by similar structures in Sanfilippo syndrome and Alzheimer's disease

06.05.2009
Rare genetic disorder may benefit from development of new dementia treatments

Researchers seeking to understand the causes of a rare genetic lysosomal storage disease, Sanfilippo syndrome type B, were surprised to find protein aggregates, known as neurofibrillary tangles, that are usually seen in Alzheimer's and other forms of dementia, according to a study published May 4 in the Proceedings of the National Academy of Sciences.

The discovery, in a study conducted at the Los Angeles Biomedical Research Institute (LA BioMed) and the University of California, Los Angeles (UCLA), means that the childhood dementia often seen in lysosomal storage diseases may have mechanisms similar to those found in Alzheimer's disease and other age-related dementias, which are characterized by an abnormal accumulation of the protein, P-tau.

The scientists also said these findings mean those suffering from these rare disorders could one day benefit from the abundance of research underway for the growing numbers of Alzheimer's patients.

"We were trying to determine what in the cellular environment makes certain brain regions more vulnerable to neurodegeneration, and we were surprised to find the presence of a protein, P-tau, and the corresponding aggregates similar to neurofibrillary tangles we see in Alzheimer's patients," said Stanislav L. Karsten, PhD, a corresponding author of the study and a LA BioMed investigator. "With the increasing incidence of Alzheimer's disease, we expect to see new drugs developed to prevent these neurofibrillary tangles or dissolve them. Our findings suggest those treatments could also benefit patients with this rare genetic disorder, Sanfilippo syndrome."

Sanfilippo syndrome, also known as MPS III, occurs in approximately one in 70,000 births, causing profound mental retardation, behavior problems and, frequently, death before the age of 20. It is one of nearly 40 lysosomal storage diseases caused by the body's inability to produce enzymes that break down and recycle materials in cells. Without the enzymes, materials are stored in virtually every cell of the body, causing severe damage over time. There is no treatment for Sanfilippo syndrome.

Alzheimer's disease affects 2 million to 4 million Americans, and their ranks are expected to grow to as many as 14 million by the middle of the 21st century as the population ages.

Grants from the National Institutes of Health, the Children's Medical Research Foundation, the National Alliance for Research on Schizophrenia and Depression and the Alzheimer's Association funded this research.

For copies of the study, "Sanfilippo syndrome type B, a lysosomal storage disease, is also a tauopathy," please contact Laura Mecoy, 310.546.5860, or LMecoy@issuesmanagement.com

About LA BioMed

Founded 56 years ago, LA BioMed at Harbor-UCLA Medical Center is one of the country's largest nonprofit independent biomedical research institutes. It conducts biomedical research, trains young scientists and provides community services, including childhood immunization and nutrition assistance. The institute's researchers conduct studies in such areas as cardiovascular disease, emerging infections, cancer, diabetes, kidney disease, dermatology, reproductive health, vaccine development, respiratory disorders, inherited illnesses and neonatology. LA BioMed is academically affiliated with the David Geffen School of Medicine at UCLA.

Laura Mecoy | EurekAlert!
Further information:
http://www.labiomed.org

More articles from Health and Medicine:

nachricht Researchers release the brakes on the immune system
18.10.2017 | Rheinische Friedrich-Wilhelms-Universität Bonn

nachricht Norovirus evades immune system by hiding out in rare gut cells
12.10.2017 | University of Pennsylvania School of Medicine

All articles from Health and Medicine >>>

The most recent press releases about innovation >>>

Die letzten 5 Focus-News des innovations-reports im Überblick:

Im Focus: Neutron star merger directly observed for the first time

University of Maryland researchers contribute to historic detection of gravitational waves and light created by event

On August 17, 2017, at 12:41:04 UTC, scientists made the first direct observation of a merger between two neutron stars--the dense, collapsed cores that remain...

Im Focus: Breaking: the first light from two neutron stars merging

Seven new papers describe the first-ever detection of light from a gravitational wave source. The event, caused by two neutron stars colliding and merging together, was dubbed GW170817 because it sent ripples through space-time that reached Earth on 2017 August 17. Around the world, hundreds of excited astronomers mobilized quickly and were able to observe the event using numerous telescopes, providing a wealth of new data.

Previous detections of gravitational waves have all involved the merger of two black holes, a feat that won the 2017 Nobel Prize in Physics earlier this month....

Im Focus: Smart sensors for efficient processes

Material defects in end products can quickly result in failures in many areas of industry, and have a massive impact on the safe use of their products. This is why, in the field of quality assurance, intelligent, nondestructive sensor systems play a key role. They allow testing components and parts in a rapid and cost-efficient manner without destroying the actual product or changing its surface. Experts from the Fraunhofer IZFP in Saarbrücken will be presenting two exhibits at the Blechexpo in Stuttgart from 7–10 November 2017 that allow fast, reliable, and automated characterization of materials and detection of defects (Hall 5, Booth 5306).

When quality testing uses time-consuming destructive test methods, it can result in enormous costs due to damaging or destroying the products. And given that...

Im Focus: Cold molecules on collision course

Using a new cooling technique MPQ scientists succeed at observing collisions in a dense beam of cold and slow dipolar molecules.

How do chemical reactions proceed at extremely low temperatures? The answer requires the investigation of molecular samples that are cold, dense, and slow at...

Im Focus: Shrinking the proton again!

Scientists from the Max Planck Institute of Quantum Optics, using high precision laser spectroscopy of atomic hydrogen, confirm the surprisingly small value of the proton radius determined from muonic hydrogen.

It was one of the breakthroughs of the year 2010: Laser spectroscopy of muonic hydrogen resulted in a value for the proton charge radius that was significantly...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

Event News

ASEAN Member States discuss the future role of renewable energy

17.10.2017 | Event News

World Health Summit 2017: International experts set the course for the future of Global Health

10.10.2017 | Event News

Climate Engineering Conference 2017 Opens in Berlin

10.10.2017 | Event News

 
Latest News

Terahertz spectroscopy goes nano

20.10.2017 | Information Technology

Strange but true: Turning a material upside down can sometimes make it softer

20.10.2017 | Materials Sciences

NRL clarifies valley polarization for electronic and optoelectronic technologies

20.10.2017 | Interdisciplinary Research

VideoLinks
B2B-VideoLinks
More VideoLinks >>>