Navigation Links
Researchers discover hereditary enzyme deficiency
Date:6/13/2012

An international research team headed by Professor Dr. Thomas Dierks at Bielefeld University has discovered a hereditary enzyme deficiency. It is a subform of the mucopolysaccharidosis syndrome and has been termed MPS IIIE or after its discoverer 'Dierks's disorder'. It leads to a progressive loss of mental abilities in mice, particularly to learning and coordination difficulties along with forgetfulness. The biochemist Dierks and his team have not only identified the disorder but also developed a treatment concept. Their findings are being published this week (CW 24) in the renowned journal 'Proceedings of the National Academy of Sciences of the USA' (PNAS).

Enzymes control the constitution and degradation of nutrient and messenger substances in the human body. If the body produces a defective enzyme due to an inherited deficiency, this control breaks down. The individual becomes ill because, for example, substances can no longer be degraded and accumulate in the body.

Dierks and his colleagues have discovered that a deficiency of the enzyme arylsulfatase G (ARSG) triggers the disease MPS IIIE in mice. Actually, the enzyme among others - is responsible for degrading the carbohydrate heparan sulfate. This process occurs within the cells inside the lysosomes. These 'recycling plants' in the cells break down no longer needed heparan sulfate molecules into their smallest components, which later are used to reassemble new molecules.

Heparan sulfate is a long-chain molecule. This chain can only be broken down from one end and only step by step. This process is performed by various enzymes including ARSG. If one of the enzymes is deficient because of a genetic defect, the complete degradation process comes to a stop. The molecular chains remain largely intact and accumulate more and more in the lysosome, which finally stops functioning. Then, also other substances such as proteins and lipids also accumulate because they are n
'/>"/>

Contact: Prof. Dr. Thomas Dierks
thomas.dierks@uni-bielefeld.de
49-521-106-2092
University of Bielefeld
Source:Eurekalert  

Page: 1 2 3

Related biology technology :

1. New England Biolabs Introduces Polbase, an Information Repository of Scientific Data for Polymerase Researchers
2. In new quantum-dot LED design, researchers turn troublesome molecules to their advantage
3. Multidisciplinary team of researchers develop world’s lightest material
4. Researchers shrink tumors and minimize side effects using tumor-homing peptide to deliver treatment
5. Innovative MetaMorph® NX Software Shatters Barriers Between Researchers and Image Analysis Goals with Exclusive Visual Workflow
6. UCLA researchers demonstrate fully printed carbon nanotube transistor circuits for displays
7. Penn and Brown researchers demonstrate earthquake friction effect at the nanoscale
8. Two Top Biological Imaging Centers Offer Powerful Free Online Tool to Researchers, Educators, and Public
9. Researchers develop one of the worlds smallest electronic circuits
10. MU researchers identify key plant immune response in fight against bacteria
11. Researchers realize high-power, narrowband terahertz source at room temperature
Post Your Comments:
*Name:
*Comment:
*Email:
Related Image:
Researchers discover hereditary enzyme deficiency
(Date:12/19/2014)... Arlington, MA (PRWEB) December 18, 2014 ... products for ophthalmology and wound care. The National Eye ... has awarded LayerBio a Phase I SBIR grant to ... , Cataracts are the most common cause of vision ... cause of blindness worldwide. According to Dr. Ken Mandell, ...
(Date:12/19/2014)... 2014 iLab Solutions, the global leader ... Siri Bryant as the new Director of Institutional Implementations ... Implementations. These two new leadership positions were created to ... ensuring that iLab continues to meet the needs of ... years, iLab has been deployed at over 450 core ...
(Date:12/19/2014)... With only 2 weeks left of 2014, ... Biohit products . The product specials include Buy 1 ... Trade-in Program, and free pipette tips. , Sartorius mLINE ... world. They are ideal for lab technicians performing long ... ,     Full volume range of 0.1 uL to 10 ...
(Date:12/19/2014)... --  ISN has released its third annual U.S. ... publication series. With data aggregated from over eight billion ... its online contractor management platform, ISNetworld, this three-part series ... statistics. The Contractor Performance ... available in two industry specific volumes: Oil & ...
Breaking Biology Technology:LayerBio Awarded NIH Grant to Develop a Drug Delivery Device for Cataract Surgery 2iLab Solutions Announces Two New Leadership Positions to Meet the Needs of Its Growing Customer Base 2Major Pipette Distributor Pipette.com Announces Sartorius Year End Specials 2ISN Releases Third Edition of US Contractor Health & Safety Performance Indicators Publication Series 2ISN Releases Third Edition of US Contractor Health & Safety Performance Indicators Publication Series 3
... ElutraSep, Inc. today announced the commercial launch of the Cryptonite™ ... . , ... Atlanta, GA (PRWEB) ... launch of the Cryptonite™ HV Filter Module for Cryptosporidium and ...
... the cool dudes from CSI, use gel electrophoresis to separate ... DNA. A team of researchers under the leadership of Vici ... how the gel influences the movement of the DNA. The ... order to analyze the forces on the DNA. The results ...
... & Philadelphia Firms ... 14 Pennsylvania is setting the stage for future ... leader in the life science industry, Governor Edward G. ... venture capital firms that will help stimulate growth in ...
Cached Biology Technology:ElutraSep Inc. Announces the Launch of the Cryptonite™ HV Filter Module for Cryptosporidium and Giardia Testing 2DNA gripped in nanopores 2Venture Capitalists to Invest in Promising PA Life Science Companies, Governor Rendell Says 2
(Date:12/11/2014)... 2014  Data Sciences International (DSI), the global ... new series of digital telemetry implants to meet ... series, part of the PhysioTel™ Digital platform, was ... physiologic data when incorporating functional endpoints into repeat-dose ... toxicology studies has evolved from short ECG strips ...
(Date:12/10/2014)... N.C. , Dec. 9, 2014  Wake Forest Baptist ... education building for its School of Medicine. Funding for ... larger capital campaign that will be publicly launched next ... be located in the former 60 series R.J. Reynolds ... Innovation Quarter. Construction will begin immediately with plans to ...
(Date:12/10/2014)... SUNNYVALE, California , December 9, 2014 /PRNewswire/ ... Integrate with ITN,s BCARD Platform ... secure, cloud-based enterprise meeting scheduling solutions for business-to-business ... International, the world leader in mobile near-field communication ... solutions.      (Logo: http://photos.prnewswire.com/prnh/20140612/691055) , ...
Breaking Biology News(10 mins):New telemetry implants expected to change how large animal toxicology studies are conducted 2Wake Forest Baptist to Build New Medical Education Facility In Wake Forest Innovation Quarter 2Wake Forest Baptist to Build New Medical Education Facility In Wake Forest Innovation Quarter 3Wake Forest Baptist to Build New Medical Education Facility In Wake Forest Innovation Quarter 4Jifflenow And ITN International Bring Cutting-Edge Badge Scanning Technology To B2B Events 2Jifflenow And ITN International Bring Cutting-Edge Badge Scanning Technology To B2B Events 3
... to stave off the cognitive deficits that occur with ... published in the second issue for May of the ... Care Medicine. Researchers examined the effects green tea ... were intermittently deprived of oxygen during 12-hour night cycles, ...
... vast array of physical and biological systems across the ... humans, says a new analysis of information not previously ... things include earlier leafing of trees and plants over ... altitudes in the northern hemisphere; changes in bird migrations ...
... climate change has made an impact on a wide ... blooming earlier across Europe, and lakes declining in productivity ... Institute for Space Science in New York and scientists ... impacts since 1970 with rises in temperatures during that ...
Cached Biology News:Green tea compounds beat OSA-related brain deficits 2Warming climate is changing life on global scale, says new study 2NASA study links Earth impacts to human-caused climate change 2
... AviTag E. coli Biotinylation Kit, Linear Template is ... coli expression kits. (Cat. No. 3 186 148, ... rapidly generate linear expression constructs by PCR containing ... a combination of T7 RNA polymerase and prokaryotic ...
... The Vibratome Roto-Shake Genie is an ... It performs five different mixing ... and rotating/rolling, as well as a ... The precision-engineered moving portion of the ...
... monoclonal [PRMT5-21] to PRMT5 Arginine methylation ... has only recently been linked to protein ... enzymes have been identified in mammalian cells. ... essential regulatory functions by methylation of key ...
... This complete kit contains ... separations from a total of ... cells, and includes the StemSep ... necessary reagents and buffers for ...
Biology Products: