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 Laser activated gold pyramids could deliver drugs, DNA into cells without harm
24.03.2017 | Harvard John A. Paulson School of Engineering and Applied Sciences

nachricht What does congenital Zika syndrome look like?
24.03.2017 | University of California - San Diego

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: Giant Magnetic Fields in the Universe

Astronomers from Bonn and Tautenburg in Thuringia (Germany) used the 100-m radio telescope at Effelsberg to observe several galaxy clusters. At the edges of these large accumulations of dark matter, stellar systems (galaxies), hot gas, and charged particles, they found magnetic fields that are exceptionally ordered over distances of many million light years. This makes them the most extended magnetic fields in the universe known so far.

The results will be published on March 22 in the journal „Astronomy & Astrophysics“.

Galaxy clusters are the largest gravitationally bound structures in the universe. With a typical extent of about 10 million light years, i.e. 100 times the...

Im Focus: Tracing down linear ubiquitination

Researchers at the Goethe University Frankfurt, together with partners from the University of Tübingen in Germany and Queen Mary University as well as Francis Crick Institute from London (UK) have developed a novel technology to decipher the secret ubiquitin code.

Ubiquitin is a small protein that can be linked to other cellular proteins, thereby controlling and modulating their functions. The attachment occurs in many...

Im Focus: Perovskite edges can be tuned for optoelectronic performance

Layered 2D material improves efficiency for solar cells and LEDs

In the eternal search for next generation high-efficiency solar cells and LEDs, scientists at Los Alamos National Laboratory and their partners are creating...

Im Focus: Polymer-coated silicon nanosheets as alternative to graphene: A perfect team for nanoelectronics

Silicon nanosheets are thin, two-dimensional layers with exceptional optoelectronic properties very similar to those of graphene. Albeit, the nanosheets are less stable. Now researchers at the Technical University of Munich (TUM) have, for the first time ever, produced a composite material combining silicon nanosheets and a polymer that is both UV-resistant and easy to process. This brings the scientists a significant step closer to industrial applications like flexible displays and photosensors.

Silicon nanosheets are thin, two-dimensional layers with exceptional optoelectronic properties very similar to those of graphene. Albeit, the nanosheets are...

Im Focus: Researchers Imitate Molecular Crowding in Cells

Enzymes behave differently in a test tube compared with the molecular scrum of a living cell. Chemists from the University of Basel have now been able to simulate these confined natural conditions in artificial vesicles for the first time. As reported in the academic journal Small, the results are offering better insight into the development of nanoreactors and artificial organelles.

Enzymes behave differently in a test tube compared with the molecular scrum of a living cell. Chemists from the University of Basel have now been able to...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

Event News

International Land Use Symposium ILUS 2017: Call for Abstracts and Registration open

20.03.2017 | Event News

CONNECT 2017: International congress on connective tissue

14.03.2017 | Event News

ICTM Conference: Turbine Construction between Big Data and Additive Manufacturing

07.03.2017 | Event News

 
Latest News

Argon is not the 'dope' for metallic hydrogen

24.03.2017 | Materials Sciences

Astronomers find unexpected, dust-obscured star formation in distant galaxy

24.03.2017 | Physics and Astronomy

Gravitational wave kicks monster black hole out of galactic core

24.03.2017 | Physics and Astronomy

VideoLinks
B2B-VideoLinks
More VideoLinks >>>