Navigation Links
Research reveals lipids' unexpected role in triggering death of brain cells
Date:11/12/2009

um signaling.

GM1-gangliosidosis is a lysosomal storage disorder in which an essential enzyme in the lysosomes is defective and cannot break down GM1. Lysosomes serve as the cell's digestive and recycling centers, stripping proteins, fats and other molecules down to their components so they can be used to assemble new molecules. Symptoms develop in childhood, in some cases shortly after birth, and include mental retardation, seizures and other problems. The outlook for patients remains bleak.

Normally, GM1 is found in the cell membrane. But previous work from d'Azzo's laboratory has shown that in patients with GM1-gangliosidosis, this lipid accumulates in other locations within the cell, including the membrane of the endoplasmic reticulum (ER). The ER is the structure where proteins are produced. GM1 accumulation in the ER membrane depletes the ER's calcium supply, disrupting the key protein-folding process. The disruption prompts the ER to target the cell for destruction.

In the current study, investigators extended their search for answers about the neurodegeneration in GM1-gangliosidosis to the mitochondria, another intracellular compartment. Some of the mitochondria are connected to the ER via the so-called mitochondria-associated ER membranes or MAMs. MAMs function like bridges, providing a route for calcium to move out of the ER and into the mitochondria. Researchers focused on the mitochondria because one of their cellular duties is to soak up and store excess intracellular calcium, including calcium from the ER.

Using a variety of techniques, researchers identified a region within MAMs where GM1 not only accumulates but sets the stage for step two of the calcium imbalance that triggers cellular suicide. Investigators reported that in healthy cells these regions, known as glycosphingolipid-enriched microdomains, or GEMs, include tiny amounts of GM1. But in mice lacking the enzyme to break down GM1, large amounts of this li
'/>"/>

Contact: Summer Freeman
summer.freeman@stjude.org
901-595-3061
St. Jude Children's Research Hospital
Source:Eurekalert

Page: 1 2 3 4

Related biology news :

1. Researchers gather in the UK to measure the impossible
2. £7.5 million ($12.6 million) collaborations lead UK research communitys response to H1N1 pandemic
3. Carnegie Mellon researchers to develop probes to study cellular GPS
4. Escaped proteins add to hearing loss in elderly, UF researchers find
5. CBS and OMD Partner to Provide the Agency With Access to the Networks Television City Research Facility in Las Vegas
6. Researchers complete draft genome sequence for cassava
7. £3.2m ($5.4 million) research project to capture brilliance of butterfly wings
8. Monell Center joins with CAS to host Beijing meeting on taste and smell research
9. Genomic research will enable greener cleanup of military explosive test sites
10. Tags reveal white sharks have neighborhoods in the north Pacific, say Stanford researchers
11. MSU researcher: Obesity significantly cuts odds of successful pregnancy
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:7/10/2014)... in New Mexico, nearly every public golf course is ... potable water supplies. Across the U.S. Southwest as a ... treated effluent. Reusing the effluent increases the sustainability of ... alike fertilize their lawns during the growing season. The ... State University turfgrass expert has a new vision for ...
(Date:7/10/2014)... June 27, 2014  Pomerantz LLP has filed ... ("Provectus" or the "Company")(NYSE-MKT: PVCT) and certain of ... States District Court, Middle District of ... of a class consisting of all persons or ... between December 17, 2013 and May 22, 2014, ...
(Date:7/10/2014)... SPRINGS, Florida , July 1, 2014 ... wallets pave way for convenience and improved security: NXT-ID, Inc. ... Inc. (NASDAQ: GOOG ), Apple Inc. (NASDAQ: ... and MasterCard Corporation (NYSE: MA) NXT-ID, Inc., (OTCQB: NXTD) ... is pleased to announce that the second series of 30 ...
Breaking Biology News(10 mins):'Tailored' water -- the latest in lawn care 2SHAREHOLDER ALERT: Pomerantz Law Firm Announces the Filing of a Class Action against Provectus Biopharmaceuticals, Inc. and Certain Officers - PVCT 2SHAREHOLDER ALERT: Pomerantz Law Firm Announces the Filing of a Class Action against Provectus Biopharmaceuticals, Inc. and Certain Officers - PVCT 3Advantages for Digital Wallets Eliminate Need to Carry Credit Cards - Large Scale CNBC Ad Campaign for Latest Smart Wallet to Commence July 7th 2Advantages for Digital Wallets Eliminate Need to Carry Credit Cards - Large Scale CNBC Ad Campaign for Latest Smart Wallet to Commence July 7th 3Advantages for Digital Wallets Eliminate Need to Carry Credit Cards - Large Scale CNBC Ad Campaign for Latest Smart Wallet to Commence July 7th 4Advantages for Digital Wallets Eliminate Need to Carry Credit Cards - Large Scale CNBC Ad Campaign for Latest Smart Wallet to Commence July 7th 5
... The old balkanized approach to ocean management, in which ... and administered by different agencies, has failed to protect ... panel of international scientists says, and should be replaced ... The panel, organized by scientists from Duke University, ...
... new and cleaner ways to meet the country,s energy ... new fuel source. The approach has the potential to ... contributing to rural economic development and lowering greenhouse emissions. ... volumes of diesel and jet transportation fuels. One of ...
... new paper by researchers at George Mason University and the ... between the diversity of organisms at the bottom of the ... Mark D. Uhen, a geologist at Mason, says that ... of whale species living at any given time period correlates ...
Cached Biology News:Marine spatial planning: A more balanced approach to ocean management 2Marine spatial planning: A more balanced approach to ocean management 3Marine spatial planning: A more balanced approach to ocean management 4Fueling the future with fish tank residue: Sandia scientist discusses use of algae as a biofuel 2Fueling the future with fish tank residue: Sandia scientist discusses use of algae as a biofuel 3Fueling the future with fish tank residue: Sandia scientist discusses use of algae as a biofuel 4New study finds link between marine algae and whale diversity over time 2
(Date:7/14/2014)... CA (PRWEB) July 14, 2014 ... patient registry platform, announced that three PatientCrossroads-based ... dystrophies; the Phelan-McDermid Syndrome International Registry; and ... Syndrome—have been awarded contracts by the Patient-Centered ... the creation of a new health data ...
(Date:7/11/2014)... July 11, 2014 /PRNewswire-iReach/ -- Maxim Peptide, a website ... are used solely in scientific experiments, is currently celebrating ... in business, Maxim Peptide, which was created by the ... new and updated website that now features a special ... been updated with fascinating articles about peptide research, including ...
(Date:7/11/2014)... EPFL and the University of  Geneva  (UNIGE) ... P. Donoghue . The American neuroscientist is to head ... The new Wyss Center for Bio- and Neuro-Engineering, ... , has named as its director one of the ... founder of Brown University,s Institute for Brain Science, has ...
(Date:7/10/2014)... Ouagadougou, Burkina Faso (PRWEB) July 10, 2014 ... Burkina Faso, today have access to an additional ... to increase access to contraception at all levels ... combining a lower-dose formulation of a widely used ... injection system. , Burkina Faso is the first ...
Breaking Biology Technology:PatientCrossroads Fuels Development of New National Clinical Research Network 2PatientCrossroads Fuels Development of New National Clinical Research Network 3Maxim Peptide Celebrates Second Anniversary Online with an Updated Website and Special Deals Section 2John P. Donoghue is Taking the Lead at the Wyss Center and is Appointed Professor at EPFL and University of Geneva 2John P. Donoghue is Taking the Lead at the Wyss Center and is Appointed Professor at EPFL and University of Geneva 3Injectable Contraceptive Launched in Burkina Faso to Expand Choice and Address Unmet Need 2Injectable Contraceptive Launched in Burkina Faso to Expand Choice and Address Unmet Need 3Injectable Contraceptive Launched in Burkina Faso to Expand Choice and Address Unmet Need 4Injectable Contraceptive Launched in Burkina Faso to Expand Choice and Address Unmet Need 5Injectable Contraceptive Launched in Burkina Faso to Expand Choice and Address Unmet Need 6
... Chromatography and Mass Spectrometry Application Report , ... Shieh, Thermo Electron Corporation, San Jose, CA , ... Use of LC/MS n has experienced a dramatic ... screening to ADME/Tox studies. The Finnigan LCQ series ion trap mass spectrometers ...
... , Larry Simonson and Lynn ... Life Sciences Inc, Applications Laboratory, Hopkinton, Massachusetts, USA , ... Initially, work in 96 well microplates was commonly ... of plate processing has grown, automated liquid handling has become a ...
... Geoffrey Grove, Susan Ryan and Ed Alderman ... Technology Department, Caliper Life Sciences, Inc., Hopkinton, MA 01748 , ... The High Volume Head is one of four ... The architecture of the device includes an array of 96 pistons, which ...
Cached Biology Technology:Use of On-line Size Exclusion Chromatography (SEC) for Sample Clean-up in LC/MS Analyses 2Use of On-line Size Exclusion Chromatography (SEC) for Sample Clean-up in LC/MS Analyses 3Use of On-line Size Exclusion Chromatography (SEC) for Sample Clean-up in LC/MS Analyses 4384 Well Pipetting Precision and Accuracy Testing Using the Sciclone Advanced Liquid Handler 2384 Well Pipetting Precision and Accuracy Testing Using the Sciclone Advanced Liquid Handler 3384 Well Pipetting Precision and Accuracy Testing Using the Sciclone Advanced Liquid Handler 4Sciclone ALH 3000 Liquid Handler: Functional Characterization of the High Volume Head - Part 1: Plate-to-Plate Precision Testing 2Sciclone ALH 3000 Liquid Handler: Functional Characterization of the High Volume Head - Part 1: Plate-to-Plate Precision Testing 3Sciclone ALH 3000 Liquid Handler: Functional Characterization of the High Volume Head - Part 1: Plate-to-Plate Precision Testing 4Sciclone ALH 3000 Liquid Handler: Functional Characterization of the High Volume Head - Part 1: Plate-to-Plate Precision Testing 5Sciclone ALH 3000 Liquid Handler: Functional Characterization of the High Volume Head - Part 1: Plate-to-Plate Precision Testing 6