Navigation Links
How mitochondrial gene defects impair respiration, other major life functions
Date:9/24/2009

Researchers are delving into abnormal gene function in mitochondria, structures within cells that power our lives. Mitochondria are the place where energy is generated from the most basic molecules of food. Because this function is essential to life, defects in mitochondria may affect a wide range of organ systems in humans and animals.

Some names of mitochondrial disorders are Leigh's disease, MELAS syndrome and complex I deficiency. These are often severe and progressive conditions that attack brain, muscles and numerous other parts of the body.

Mitochondrial diseases are individually very rare, but because hundreds of them exist, they collectively have a large impact, affecting at least 1 in 5,000 people, and perhaps more, who often remain undiagnosed. In addition to a wide array of diseases originating in the mitochondria itself, malfunctioning mitochondria also contribute to complex disorders like Parkinson's disease, Alzheimer's disease, epilepsy and diabetes, among others.

For such crucial biological actors, much remains unknown about exactly how mitochondria function. A new study, published Aug. 12 in the online journal PLoS One, sheds light on mitochondrial biology.

Using genetic engineering, researchers interrupted the activity of individual genes directly involved in the production of energy within mitochondria. "If we knock down the function of specific system components, what happens?" said study leader Marni J. Falk, M.D., who directs the Mitochondrial-Genetics Disease Clinic at The Children's Hospital of Philadelphia. "Our ultimate goal is to translate the knowledge into targeted therapies, that is, effective ways to intervene. But first we need to understand the underlying disease mechanisms."

Falk's team made use of a simple model organism often studied in biology, Caenorhabditis elegans, which is a small worm called a nematode. Because mitochondria arose very early in evolution and play such
'/>"/>

Contact: John Ascenzi
Ascenzi@email.chop.edu
267-426-6055
Children's Hospital of Philadelphia
Source:Eurekalert

Page: 1 2 3

Related biology news :

1. Large reservoir of mitochondrial DNA mutations identified in humans
2. Mitochondrial cholesterol makes response to chemotherapy difficult in hepatic cancer
3. Hebrew University develops novel approach for treating mitochondrial disorders
4. Mitochondrial dysfunction and redox signaling in atrial tachyarrhythmia
5. Zebrafish to shed light on human mitochondrial diseases
6. Scientists show that mitochondrial DNA variants are linked to risk factors for type 2 diabetes
7. Essential nutrient found in eggs may help lower risk of neural tube defects
8. Embryology study offers clues to birth defects
9. Folic acid to prevent congenital heart defects
10. Low levels of vitamin B12 may increase risk for neural tube defects
11. Case Western Reserve University uncovers genetic basis for some birth defects
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:3/12/2015)... -- The mascots of the Atlantic Coast Conference spent ... Forest Baptist Medical Center. They stopped by as part ... Basketball Tournament. Young patients enjoyed bonding with their favorite ... Brenner Children,s Hospital is the pediatric arm ... hospital in northwest North Carolina ...
(Date:3/11/2015)... --Research and Markets ( http://www.researchandmarkets.com/research/3p32qm/access_control ) has ... by Product, Application & By Geography - Global Forecast ... , This report predicts that the access ... 2020, with an estimated CAGR of 10.6%. ... such as contact cards & readers, contactless cards & ...
(Date:3/10/2015)... -- Transforming How We Look at Medicine ... Human Space Flight" by Michael A. Schmidt ... was recently featured by Springer Science Media, highlighting the most ... Specifically, "Personalized Medicine in Human Space Flight" was among ... and 2014 from Springer in the journal Metabolomics ...
Breaking Biology News(10 mins):Global Access Control (Cards & Readers, Biometrics, Controllers, Management Software) Market 2015-2020 - Analysis of the $10 Billion Market 2"Personalized Medicine in Human Space Flight" Listed Among Most Influential Papers of 2013 and 2014 2
... efforts may do it more for themselves than the ... Alberta case study shows. A study of purple ... their landrevealed that they were more motivated to take ... altruistic reasons. "Though there were areas of overlap, ...
... research to disease cure isn,t usually a smooth one. ... that of the "clinician-scientist" a doctor who understands disease ... Yet an editorial published in Disease Models & Mechanisms ... UK and elsewhere are not prospering, but rather are "under ...
... (IAH) are stepping up the battle against the devastating ... ways to trap and monitor midges with cutting edge ... gaining an understanding of how the insects spread the ... advise farmers how and when to protect their animals. ...
Cached Biology News:For the birds or for me? Why do conservationists really help wildlife? 2Medical doctors who do research could be a dying breed 2Midge-hunting scientists tackle spread of devastating bluetongue virus 2Midge-hunting scientists tackle spread of devastating bluetongue virus 3
(Date:4/1/2015)... and NEW YORK CITY ... -- As a leading provider of market research reports and ... Worldwide, LLC, indicating that 2015 will be a year of ... For executives operating a stem cell company or cord ... difficult task. You must analyze market conditions, predict industry trends, ...
(Date:4/1/2015)... Earlier this year, A.J. O’Connor Associates ... that represents research-based life science organizations in New Jersey, ... Vice President of Business Development & Executive Coach, attended ... attracted over 750 attendees. , In one of ... cancer survivor shared his moving story of how, almost ...
(Date:4/1/2015)... April 1, 2015 Immunocore ... novel biological drugs to treat cancer, viral infections ... the appointment of Christina Coughlin as ... for providing medical and regulatory leadership to the ... Dr. Coughlin has extensive experience in oncology ...
(Date:3/31/2015)... , March 31, 2015  Biotest Pharmaceuticals Corporation ... pleased to announce the addition of its newest plasma ... 205 Conway, Arkansas . The over ... for business on March 26 th 2015 and ... to eighteen. "This facility represents another ...
Breaking Biology Technology:MarketResearch.com: "Blockbuster" Year for Stem Cells Creates Market Opportunities, Says BioInformant Worldwide Report 2MarketResearch.com: "Blockbuster" Year for Stem Cells Creates Market Opportunities, Says BioInformant Worldwide Report 3MarketResearch.com: "Blockbuster" Year for Stem Cells Creates Market Opportunities, Says BioInformant Worldwide Report 4HR Consulting Firm, A.J. O'Connor Associates Joins BioNJ – The Vibrant 400 Member Strong Life Sciences Industry Association - and Attends its Annual Dinner 2Immunocore Appoints Dr. Christina Coughlin as Chief Medical Officer 2Immunocore Appoints Dr. Christina Coughlin as Chief Medical Officer 3Immunocore Appoints Dr. Christina Coughlin as Chief Medical Officer 4Biotest Pharmaceuticals Corporation Announces the Opening of its Newest Plasma Collection Facility in Conway, Arkansas 2
... employees enjoy after work social and pledge drive , , ... Americas Restaurant on The Waterway in ... Oncology, Inc . delighted in an after work social and ... . , , (Photo: http://www.newscom.com/cgi-bin/prnh/20090605/DA28382 ) , ...
... Johnson & Johnson (NYSE: JNJ ), through a ... a cash tender offer to purchase all outstanding shares of ... ). Johnson & Johnson reported on May 21, 2009, ... Upon the successful closing of the tender offer, shareholders of ...
... (Nasdaq: DNDN ) today announced that management will present at ... Goldman Sachs 30th Annual Global Healthcare Conference, Tuesday, June ... Annual Needham Life Sciences Conference, Thursday, June 11, 2009, ... be audio webcast live and available for replay from Dendreon,s website, ...
Cached Biology Technology:Americas on the Waterway Supports US Oncology Foundation 2Americas on the Waterway Supports US Oncology Foundation 3Johnson & Johnson Begins Tender Offer to Acquire Cougar Biotechnology 2Johnson & Johnson Begins Tender Offer to Acquire Cougar Biotechnology 3Johnson & Johnson Begins Tender Offer to Acquire Cougar Biotechnology 4
One-step, microplate or cuvet, colorimetric, linear detection range 0.7 mg/dL to 35 mg/dL. Procedure: 5 min....
[5,6,8,9,12,14,15(n)-3H]Prostaglandin D2, 925 kBq, 25 uCi. Methanol:water:acetonitrile (3:2:1) solution.> 3.0 TBq/mmol, > 80 Ci/mmol.3.7 MBq/ml, 100 uCi/ml. Category: Radiochemicals &Radiation Safety...
One-step, microplate or cuvet, turbidometric, linear detection range 0.096 mg/dL to 11.5 mg/dL. Procedure: 5 min....
Moue Serum 8-12 Weeks Old...
Biology Products: