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:7/11/2014)... Social animals often develop relationships with other group members ... In wild chacma baboons the strategy for grooming activities ... are just published in the scientific journal Biology ... of baboons is not practiced without ulterior motives. To ... for the individual, while grooming another individual can provide ...
(Date:7/11/2014)... 11, 2014 The increased risk of kidney injury ... resuscitation fluids reflects the mass of HES molecules, according ... official journal of the International Anesthesia Research Society (IARS). ... harmful effect of HES on cultured human renal proximal ... Christian Wunder and colleagues of University Hospital Wrzburg, Austria. ...
(Date:7/11/2014)... cannabinoid receptor type 1 can inhibit voltage-gated ... reduce neurotransmitter release. However, some scholars demonstrated ... extracellular Ca2+ influx and increase neurotransmitter release. ... Hospital Affiliated to Tongji Medical College, Huazhong ... whole cell voltage-clamp and calcium imaging in ...
Breaking Biology News(10 mins):Baboons groom early in the day to get benefits later 2In lab studies, hydroxyethyl starch has direct harmful effects on kidney cells 2
... More than 20 percent of atheist scientists are spiritual, ... public marries spirituality and religion, the study found that spirituality ... with scientific discovery for "spiritual atheist" scientists. ... Sociology of Religion . Through in-depth interviews ...
... , A new study from Cambridge University has for ... a higher than average number of autistic traits. ... today in the Journal of Autism and Developmental ... of biological girls with gender incongruence that persists into ...
... Amsterdam, 5 May 2011 Elsevier, the ... products and services, announced today the launch ... The journal aims at reporting the most recent ... intelligent computation based on the principles of swarm ...
Cached Biology News:More than 20 percent of atheist scientists are spiritual 2Female-to-male transsexual people have more autistic traits 2Elsevier announces launch of Swarm and Evolutionary Computation 2
(Date:7/10/2014)... 2014 On July 9 Prime ... presented Russia,s first national "Industry" ... Company is developing a unique project called MabNext ... a number of innovative drugs based on monoclonal antibodies ... took place at the International Exhibition "Innoprom 2014" in ...
(Date:7/10/2014)... 10, 2014   Ceres, Inc . (Nasdaq: ... announced today financial results for the three months ended ... business. Ceres reported that the company and ... this growing season in Brazil, which concluded in June, ... sorghum evaluation areas for part of the growing season. ...
(Date:7/10/2014)... in the journal PNAS (Proceedings of the National ... day and sleep deprivation have a significant effect on ... at the best time of day to test for ... administering medicines effectively. , Researchers from the University of ... the links between sleep deprivation, body clock disruption and ...
(Date:7/10/2014)... can sit upside down forever. Nanophysics makes sure of that. ... article just published in Physical Review E . Knowledge ... spiders that seem to be able to sit still forever, ... many years. We will soon be able to buy smart ... foot. But the fact is, sooner or later the grip ...
Breaking Biology Technology:Dmitry Medvedev Presented BIOCAD the First National "Industry" Award 2Ceres Announces Fiscal Third Quarter 2014 Financial Results 2Ceres Announces Fiscal Third Quarter 2014 Financial Results 3Ceres Announces Fiscal Third Quarter 2014 Financial Results 4Ceres Announces Fiscal Third Quarter 2014 Financial Results 5Ceres Announces Fiscal Third Quarter 2014 Financial Results 6Ceres Announces Fiscal Third Quarter 2014 Financial Results 7Ceres Announces Fiscal Third Quarter 2014 Financial Results 8Ceres Announces Fiscal Third Quarter 2014 Financial Results 9Time of day crucial to accurately test for diseases, new research finds 2Even geckos can lose their grip 2
... , ROCKAWAY, N.J., Dec. 14 ... Approach , published by the Infusion Nurses ... extravasation.(1) Anthracyclines are a group of chemotherapy medications including ... the treatment of various types of cancer since the early ...
... , WALTHAM, Mass., Dec. ... that it has signed a new, three-year master services ... and services that serve patients in approximately 100 countries. ... for translation of the company,s online content, which includes ...
... WALTHAM, Mass., Dec. 14 Decision Resources, one of the world,s ... that, owing to the launches of several new agents, the atrial ... in 2008 to $6.1 billion in 2018 in the United States, ... new Pharmacor report entitled Atrial Fibrillation finds that a ...
Cached Biology Technology:Infusion Nurses Society References Totect(R) (Dexrazoxane For Injection) as the Only Antidote for the Treatment of Anthracycline Extravasation 2Infusion Nurses Society References Totect(R) (Dexrazoxane For Injection) as the Only Antidote for the Treatment of Anthracycline Extravasation 3Infusion Nurses Society References Totect(R) (Dexrazoxane For Injection) as the Only Antidote for the Treatment of Anthracycline Extravasation 4Infusion Nurses Society References Totect(R) (Dexrazoxane For Injection) as the Only Antidote for the Treatment of Anthracycline Extravasation 5Multi-Billion Dollar Biotech Firm Chooses Lionbridge as Exclusive Translation Provider with Three-Year Master Services Agreement 2Multi-Billion Dollar Biotech Firm Chooses Lionbridge as Exclusive Translation Provider with Three-Year Master Services Agreement 3The Atrial Fibrillation Drug Market Will Increase More Than Sevenfold, from $790 Million in 2008 to $6.1 Billion in 2018 2
Detect and measure levels of mouse IgG2b antibody isotypes quickly and easily....
Fast, sensitive quantitation of Mouse IgM concentration....
Detect and measure levels of mouse IgG3 antibody isotypes quickly and easily....
Request Info...
Biology Products: