Navigation Links
Severe congenital disorder successfully treated in a mouse model for the first time
Date:12/22/2011

ached to the body's proteins or in some cases, are not attached at all. The lack of oligosaccharide chains leads to impairment of neurological, growth and organ development. The disorder only manifests if the baby inherits a mutated gene from both the mother and the father. The parents, who each carry one mutated and one "healthy" copy of the gene, do not exhibit any symptoms.

Mice take up mannose in drinking water

The mouse model developed by Prof. Krner and his team is characterized by mutations in the Phosphomannomutase 2 gene and demonstrates reduced enzyme activity, comparable to CDG-Ia in man. In their current study, the scientists exploited the ability of mannose to cross the placental barrier. This means that if the pregnant mouse takes up mannose, it also reaches the embryos in the uterus.

"One week prior to mating, we began giving the female mice mannose with their drinking water," explained biochemist Prof. Krner. The additional mannose supply up to birth increased the mannose levels in the embryos' blood. "The mice were born without defects and also after they were born, developed without any symptoms of the disorder, even if they no longer took up any mannose," Krner added. The successful studies performed by the Heidelberg University Hospital researchers clearly show the key role played by the supply of proteins with sugar chains during embryonic development.

New therapeutic approach

"Clinical studies in the U.S. and Germany have already been performed in which children with CDG-Ia were given mannose after they were born, either orally or by intravenous infusion. Unfortunately, these attempts have not been successful," explained Dr. Christian Thiel, head of the laboratory. "This means that the critical point at which it is possible to influence development must be during development in the uterus." For women with a risk of CDG-Ia, administering mannose during pregnancy may serve as a new therap
'/>"/>

Contact: Prof. Dr. rer. nat. Christian Krner
christian.koerner@med.uni-heidelberg.de
49-622-156-39993
University Hospital Heidelberg
Source:Eurekalert  

Page: 1 2 3

Related biology news :

1. Severe climate change costs forecast for Pennsylvania, N.C., Tennessee, N.D.
2. Franklin Square to study airway bypass procedure for severe emphysema
3. Severe gestational hypertension may protect against testicular cancer
4. UT Public Health researchers find link to severe Staph infections
5. Preclinical work shows how one gene causes severe mental retardation
6. New center at Georgia Tech aims to improve recovery of soldiers with severe injuries
7. UF makes gene therapy advance in severe genetic disorder
8. Smaller than expected, but severe, dead zone in Gulf of Mexico
9. Research shows rates of severe childhood obesity have tripled
10. Severe breathing disorders during sleep are associated with an increased risk of dying
11. Test could predict which idiopathic pulmonary fibrosis patients will become severely ill
Post Your Comments:
*Name:
*Comment:
*Email:
Related Image:
Severe congenital disorder successfully treated in a mouse model for the first time
(Date:11/3/2014)... , Nov. 3, 2014  NeuroSigma ® ... -based life sciences company focused on commercialization of ... for the treatment of neurological and neuropsychiatric ... Industrial Property has issued a Notice of Allowance ... application is co-owned by NeuroSigma and the Regents ...
(Date:11/2/2014)... issue of Physics World , James Dacey explores ... of death" to take their innovations from the lab ... gruelling challenge facing all start-up companies as they move ... because of two main factors: physics-based inventions are usually ... turn out to be a lot more complicated than ...
(Date:11/2/2014)... to put a flashlight up to our palms to ... in St. Louis engineers are using a similar idea ... imaging of cancerous tissues and to develop potential treatments. ... Distinguished Professor of Biomedical Engineering at the School of ... technology that allows researchers to better focus light in ...
Breaking Biology News(10 mins):NeuroSigma Receives Notice of Allowance from the Mexican Institute of Industrial Property Covering Subcutaneous Trigeminal Nerve Stimulation (sTNS) 2NeuroSigma Receives Notice of Allowance from the Mexican Institute of Industrial Property Covering Subcutaneous Trigeminal Nerve Stimulation (sTNS) 3The 'valley of death' facing physics start-ups 2Improving imaging of cancerous tissues by reversing time 2
... KAN. -- It,s a cloak that surpasses all others: ... change the way bacteria and other cells are imaged. ... Kansas State University, and his research team are wrapping ... bacteria under electron microscopes. Berry,s method creates a carbon ...
... 15In the latest twist on optical knots, New York ... create extended and knotted optical traps in three dimensions. ... Optical Society,s (OSA) open-access journal Optics Express , ... intensity traces out a knotted trajectory in space, for ...
... variety of plant seedlings suffer most from competition when planted ... relatives in field soils, researchers from Case Western Reserve University ... when seeds of the same species are buried among relatives ... and grow better early in life in close relatives, habitats ...
Cached Biology News:All wrapped up: K-State researcher's graphene cloak protects bacteria, leading to better images 2All wrapped up: K-State researcher's graphene cloak protects bacteria, leading to better images 3Tying the knot with computer-generated holograms: Winding optical path moves matter 2Tying the knot with computer-generated holograms: Winding optical path moves matter 3Seedlings thrive with distant relatives, seeds with close family 2
(Date:11/21/2014)... 2014 Deep Research Report on ... professional and in-depth research report on the Sodium ... like its definition, classification, application, and industry chain ... covers the international market analysis, including China’s domestic ... US, Europe, Asia, China, Japan etc. regions) industry ...
(Date:11/21/2014)... /PRNewswire/ - BIOREM Inc. (TSXV: BRM) ("Biorem" or "the Company") today announced ... 30, 2014.  Biorem,s complete 2014 third quarter financial statements and MD&A ... Summary:Three-months ended September 30, , Nine-months ended ... 2014 , 2013 , 2014 ... , 6,715 , 14,476 Gross profit , ...
(Date:11/18/2014)... Athena Signature Series: Turning Cancer ‘Off’ – featuring Dr. ... in the Regulatory Biology Laboratory at the Salk Institute, studies ... an “off switch” for drug resistance in cancer. , Learn ... is making a global impact in the fight against cancer. ... Cheryl K. Goodman, CEO of Social Global Mobile LLC, for ...
(Date:11/18/2014)... 18, 2014 Array Architects is proud ... expertise to the nation's hospitals and health systems. It ... those dedicating their careers to the advancement of both ... in her respective field, Laura Silvoy has been chosen ... 2014 Winter Simulation Conference. , Laura will present ...
Breaking Biology Technology:Sodium Ferrocyanide Market: Analysis for Global & China Regions Development Trends Available at DeepResearchReports.com 2Sodium Ferrocyanide Market: Analysis for Global & China Regions Development Trends Available at DeepResearchReports.com 3Biorem Reports Third Quarter Results 2Biorem Reports Third Quarter Results 3Array Healthcare Simulation Expert Slated to Speak at Winter Simulation Conference 2
... Fourth Quarter Highlights:, -- Record revenues of $82.0 million, up 22% from the prior year,s fourth ... quarter ... $0.23, a 130% increase over last year,s ... of $24.3 million, revolving debt facility paid off, -- Launch of four new food and beverage products and ...
... WILMINGTON, N.C., Dec. 12 PPD, Inc. ... M.D., as director,of strategic development, to lead the ... Dr. Mathew will provide strategic leadership for PPD,s ... project management,and patient recruitment for key therapeutic areas. ...
... 12 Beckman Coulter,Inc. (NYSE: BEC ), ... complex biomedical testing, announced today that Susan R.,Nowakowski, ... Services, Inc. (NYSE: AHS ) has been ... effective December 10., (Logo: http://www.newscom.com/cgi-bin/prnh/20031202/BECLOGO ...
Cached Biology Technology:Martek Announces Fourth Quarter and FY 2007 Financial Results 2Martek Announces Fourth Quarter and FY 2007 Financial Results 3Martek Announces Fourth Quarter and FY 2007 Financial Results 4Martek Announces Fourth Quarter and FY 2007 Financial Results 5Martek Announces Fourth Quarter and FY 2007 Financial Results 6Martek Announces Fourth Quarter and FY 2007 Financial Results 7Martek Announces Fourth Quarter and FY 2007 Financial Results 8Martek Announces Fourth Quarter and FY 2007 Financial Results 9Martek Announces Fourth Quarter and FY 2007 Financial Results 10Martek Announces Fourth Quarter and FY 2007 Financial Results 11Martek Announces Fourth Quarter and FY 2007 Financial Results 12Martek Announces Fourth Quarter and FY 2007 Financial Results 13Martek Announces Fourth Quarter and FY 2007 Financial Results 14Martek Announces Fourth Quarter and FY 2007 Financial Results 15Mathew to Lead PPD Drug Development Operations and Expansion in India 2Mathew to Lead PPD Drug Development Operations and Expansion in India 3Beckman Coulter Elects AMN Healthcare Executive to Board of Directors 2
Molecular Biological Resources offers bulk quantities of existing protein products. Please see the Chimerx divisions website for existing products and contact lloconne@molbiores.com for pricing....
... Quality ISO 9001:2000 Certified USDA ... Precedures Validated, SOP's Training AALAS ... for All Procedures GLP Documentation Upon Request ... stop shopping point for all your custom ...
... XL provides balance data collection and automation that ... integration with Microsoft Excel , Pre-configured library ... , New balances can be added and configured ... from multiple balances into one spreadsheet ...
Peptide-affinity Purified Polyclonal Antibody to Sphingolipid Receptor Edg8/S1P5...
Biology Products: