Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:

 

Flies offer insight into human metabolic disease

01.06.2010
Commonalities between flies and humans make a valuable new model for galactosemia
Galactosemia is a metabolic disease resulting from an inherited defect that prevents the proper metabolism of galactose, a sugar commonly found in dairy products, like milk. Exposure of affected people to galactose, can damage most of their organ systems and can be fatal. The ability to study the disease is limited by a lack of animal models. New information suggests that similarities between humans and flies may provide scientists with useful clues.

The inability to breakdown simple sugars in common foods, such as milk, can lead to the accumulation of sugars in the blood, which become toxic and damaging to a variety of organ systems. People with galactosemia, either classic galactosemia or epimerase deficiency galactosemia, have genetic mutations that decrease their levels of the key enzymes (GALT and GALE) responsible for the metabolism of a common form of dietary sugar. Without proper levels of these proteins, these people are unable to process the sugar, galactose, which makes up about half of the calories in milk. Both disorders can have severe effects. Patients suffer from liver and brain damage, cataracts, and kidney failure. The disease can be fatal. There is currently no cure and prognosis and treatment remain ill-defined, partly due to the lack of a good animal model that scientists can use to study the disease and to develop potential treatments.

Discovery of treatments for galactosemia is complicated by the unique sensitivities among different organisms to defects in sugar metabolism. For example, galactose accumulation in mice does not have the same physiological consequences as it does in humans, limiting the applicability of mouse models and slowing advances in this area of research. The fly (Drosophila melanogaster) is a popular laboratory model organism that has been used for many decades and in numerous studies, including those of human metabolic disease.

Scientists at Emory University developed flies that carry genetic changes similar to those found in patients with galactosemia. Like patients with classic galactosemia, flies that are missing GALT survive if they are raised on food that does not contain galactose, but die in development if exposed to high levels of galactose. Flies with impaired GALE function also succumb in greater numbers when exposed to galactose during development, similar to patients with defects in the same area of their metabolic pathway. The Emory scientists also tested the relationship between the timing of galactose exposure with the fly's outcome, and designed and characterized flies in which they could remove or control the production of GALT or GALE at variable points in the animal's development to determine when and where the sugar breakdown was most needed. These models can help researchers understand how changes in sugar metabolism lead to disease and open the door to novel drug discovery by serving as a testing ground for candidate therapeutics.

This work is presented in corresponding Research Articles in Volume 3 issue 7/8 of the research journal, Disease Models & Mechanisms (DMM), , published by The Company of Biologists, a non-profit organisation based in Cambridge, UK. The first article entitled 'A Drosophila melanogaster model of classic galactosemia, was written Rebekah Kushner, Emily Ryan, Jennifer Sefton, Rebecca Sanders, Patricia Jumbo Lucioni, Kenneth Moberg, and Judith Fridovich-Keil. The second article entitled 'UDP-galactose 4' epimerase (GALE) is essential for development of Drosophila melanogaster' was written by Rebecca Sanders, Jennifer Sefton, Kenneth Moberg, and Judith Fridovich-Keil

About Disease Models & Mechanisms:

Disease Models & Mechanisms (DMM) (http://dmm.biologists.org) is a new research journal, launched in 2008, that publishes primary scientific research, as well as review articles, editorials, and research highlights. The journal's mission is to provide a forum for clinicians and scientists to discuss basic science and clinical research related to human disease, disease detection and novel therapies. DMM is published by the Company of Biologists, a non-profit organization based in Cambridge, UK.

The Company also publishes the international biology research journals Development, Journal of Cell Science, and The Journal of Experimental Biology. In addition to financing these journals, the Company provides grants to scientific societies and supports other activities including travelling fellowships for junior scientists, workshops and conferences. The world's poorest nations receive free and unrestricted access to the Company's journals.

Kristy Kain | EurekAlert!
Further information:
http://dmm.biologists.org

More articles from Life Sciences:

nachricht Closing the carbon loop
08.12.2016 | University of Pittsburgh

nachricht Newly discovered bacteria-binding protein in the intestine
08.12.2016 | University of Gothenburg

All articles from Life Sciences >>>

The most recent press releases about innovation >>>

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

Im Focus: Significantly more productivity in USP lasers

In recent years, lasers with ultrashort pulses (USP) down to the femtosecond range have become established on an industrial scale. They could advance some applications with the much-lauded “cold ablation” – if that meant they would then achieve more throughput. A new generation of process engineering that will address this issue in particular will be discussed at the “4th UKP Workshop – Ultrafast Laser Technology” in April 2017.

Even back in the 1990s, scientists were comparing materials processing with nanosecond, picosecond and femtosesecond pulses. The result was surprising:...

Im Focus: Shape matters when light meets atom

Mapping the interaction of a single atom with a single photon may inform design of quantum devices

Have you ever wondered how you see the world? Vision is about photons of light, which are packets of energy, interacting with the atoms or molecules in what...

Im Focus: Novel silicon etching technique crafts 3-D gradient refractive index micro-optics

A multi-institutional research collaboration has created a novel approach for fabricating three-dimensional micro-optics through the shape-defined formation of porous silicon (PSi), with broad impacts in integrated optoelectronics, imaging, and photovoltaics.

Working with colleagues at Stanford and The Dow Chemical Company, researchers at the University of Illinois at Urbana-Champaign fabricated 3-D birefringent...

Im Focus: Quantum Particles Form Droplets

In experiments with magnetic atoms conducted at extremely low temperatures, scientists have demonstrated a unique phase of matter: The atoms form a new type of quantum liquid or quantum droplet state. These so called quantum droplets may preserve their form in absence of external confinement because of quantum effects. The joint team of experimental physicists from Innsbruck and theoretical physicists from Hannover report on their findings in the journal Physical Review X.

“Our Quantum droplets are in the gas phase but they still drop like a rock,” explains experimental physicist Francesca Ferlaino when talking about the...

Im Focus: MADMAX: Max Planck Institute for Physics takes up axion research

The Max Planck Institute for Physics (MPP) is opening up a new research field. A workshop from November 21 - 22, 2016 will mark the start of activities for an innovative axion experiment. Axions are still only purely hypothetical particles. Their detection could solve two fundamental problems in particle physics: What dark matter consists of and why it has not yet been possible to directly observe a CP violation for the strong interaction.

The “MADMAX” project is the MPP’s commitment to axion research. Axions are so far only a theoretical prediction and are difficult to detect: on the one hand,...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

Event News

ICTM Conference 2017: Production technology for turbomachine manufacturing of the future

16.11.2016 | Event News

Innovation Day Laser Technology – Laser Additive Manufacturing

01.11.2016 | Event News

#IC2S2: When Social Science meets Computer Science - GESIS will host the IC2S2 conference 2017

14.10.2016 | Event News

 
Latest News

Closing the carbon loop

08.12.2016 | Life Sciences

Applicability of dynamic facilitation theory to binary hard disk systems

08.12.2016 | Physics and Astronomy

Scientists track chemical and structural evolution of catalytic nanoparticles in 3-D

08.12.2016 | Materials Sciences

VideoLinks
B2B-VideoLinks
More VideoLinks >>>