Navigation Links
Flies offer insight into human metabolic disease
Date:5/31/2010

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.


'/>"/>

Contact: Kristy Kain
kristy.kain@vanderbilt.edu
615-343-1298
The Company of Biologists
Source:Eurekalert

Related medicine news :

1. The buzz on fruit flies: New role in the search for addiction treatments
2. Latest epidemic? High cholesterol, obesity in fruit flies
3. AthletiCo Physical Therapy Enhances Work Rehabilitation Program by Offering Ergonomics and Job Analysis
4. U of A discovery offers promising research for spinal-cord injury treatments
5. Khannna Institute to offer Crystalens in Quarter Diopter Steps
6. Home Gym Equipment Supplier, Fitness 4 Home Online, Features Unique Fitness Offerings
7. Scientists offer solutions to arsenic groundwater poisoning in southern Asia
8. Robotic-assisted vasectomy reversal offers greater chance of fatherhood
9. As Popularity of Dental Implants Rise, a Chicago Cosmetic Dentistry Practice's On-site Lab Offers Patients a Unique Incentive
10. Chef Charles Phillips of Nashville's 1808 Grille, Teams up with National Foundation for Cancer Research, Offering Gourmet Healthy Living Options
11. Breast Cancer Study Offers New Hope
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:5/26/2017)... ... May 26, 2017 , ... Water damage to the flooring of several ... had left education officials with a number of critical issues to address before students ... to be accomplished with little or no disruption to class schedules. Second, the project ...
(Date:5/24/2017)... ... May 24, 2017 , ... In modern research, success depends ... instruments for research and understanding the basic principles that were designed to drive ... innovations in stereo microscopy for brightfield and fluorescence typically used in laboratories working ...
(Date:5/24/2017)... ... 24, 2017 , ... Altec Products, Inc., a leader in enterprise ... one-day technology conference in San Diego, CA. , At nVerge 2017, Altec will ... utilize and enhance their Sage ERP solutions by providing improved visibility and control to ...
(Date:5/24/2017)... ... May 24, 2017 , ... Drs. ... NY, who have now spent 10 years as clinical instructors for the reputable ... College of Dentistry. Through the program, private practitioners receive cutting-edge clinical training and ...
(Date:5/24/2017)... ... , ... Patients who want to receive cosmetic dentistry procedures such as Invisalign® ... for a consultation, with or without a referral. Dr. Bedich enjoys improving the appearance ... , Dr. Bedich offers a variety of cosmetic dentistry services at his practice that ...
Breaking Medicine News(10 mins):
(Date:5/10/2017)... MARLBOROUGH, Mass., May 10, 2017 Hologic, Inc. ... financial results for the fiscal second quarter ended April ... (EPS) of $1.84 increased 666.7% compared to the prior ... business resulted in a significant gain, while non-GAAP diluted ... increased 3.2%, or 3.8% in constant currency terms.  Excluding ...
(Date:5/10/2017)... Md. , May 10, 2017 CSSi, ... solutions for the clinical research industry, is proud to ... www.CSSiEnroll.com . The new website features both enriched content ... user experience and enhances the company,s already well-established position ... industry. "After many months of hard ...
(Date:5/9/2017)... 9, 2017  Semler Scientific, Inc. (OTCQB: SMLR), ... to improve the clinical effectiveness and efficiency of ... first quarter ended March 31, 2017. ... customers to identify when preventive care options are ... like heart attacks or strokes occur," said ...
Breaking Medicine Technology: