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:10/25/2014)... October 24, 2014 Today, Nerium International, ... the winners of its “Reflect Your Youth” contest, an ... only seen a difference in their skin since using ... new outlook on life as a result of their ... as an effort by Nerium International to inspire conversations ...
(Date:10/25/2014)... Durham, NC (PRWEB) October 24, 2014 ... appears to overcome several pitfalls of current skin grafting ... scarring and produces hair. The method, outlined in the ... a breakthrough for treating deep skin injuries that result ... skin injury completely destroys the skin’s regenerative elements. These ...
(Date:10/25/2014)... October 24, 2014 Oramed Pharmaceuticals ... company focused on the development of oral drug delivery ... Kidron will present at FireRock Capital,s Micro-Cap Conference. ... Micro-Cap Conference ,Date: Tuesday, October 28, 2014 ,Time: 9:15am ... New York City , About ...
(Date:10/25/2014)... Oct. 24, 2014  RegeneRx Biopharmaceuticals, Inc. (OTCBB: RGRX) ("the ... the Fourth International Symposium on Thymosins in Health and ... "When thymosin beta 4 (TB4) linked with ... peritoneal cavity in a rodent model of stroke, it ... brain parenchyma (the functional tissue of the brain).   The ...
Breaking Biology Technology:Nerium International™ Inspires Conversation About the Journey of Aging with “Reflect Your Youth” Contest 2New engineering method shows promise for faster healing, more cosmetically appealing skin grafts 2New engineering method shows promise for faster healing, more cosmetically appealing skin grafts 3Oramed to Present at the FireRock Annual Micro-Cap Conference 2Oramed to Present at the FireRock Annual Micro-Cap Conference 3Researchers Report Thymosin Beta 4 Crosses Blood-Brain Barrier In Animal Stroke Model 2
... Wash., June 16, 2011 SCOLR Pharma, Inc. (OTC: ... of $1 million principal amount of its 8% Senior ... initial closing of its private offering of up to ... offer additional Debentures, up to the aggregate maximum of ...
... KNDL ), a leading, global full-service ... senior clinical development experts will present on important ... 47th Annual Meeting, June 19-23, 2011, in Chicago. ... benefits of hybrid monitoring; finding an economic balance ...
... 2011 MacuCLEAR, Inc., a specialty clinical stage pharmaceutical ... vascular disorders of the eye, announced today that Philip ... OneMedForum New York 2011 on Thursday, June 23, 2011 ... New York City.   OneMedForum will focus ...
Cached Biology Technology:SCOLR Pharma, Inc. Announces Initial Closing of Private Placement 2SCOLR Pharma, Inc. Announces Initial Closing of Private Placement 3Kendle Experts to Address Key Industry Issues at Drug Information Association 47th Annual Meeting 2Kendle Experts to Address Key Industry Issues at Drug Information Association 47th Annual Meeting 3MacuCLEAR, Inc. to Present at OneMedForum New York 2011 and at the 2011 BIO Business Forum During the Annual BIO International Convention in Washington, D.C. 2
(Date:10/19/2014)... Improving household electricity access in India over the ... nation,s total carbon emissions growth during that time, ... journal Nature Climate Change ., "Energy access ... all aspects of life, including education, communication, and ... the study. , While increased energy access is ...
(Date:10/17/2014)... Rockville, MD – Accurate knowledge regarding Ebola is critical ... current risk of hazardous global outbreak and epidemic. The ... has launched a special issue, Ebola Virus and Public ... with necessary knowledge in this critical societal moment. ... Primer on Ebola for Clinicians . The primer was ...
(Date:10/16/2014)... Cancer constantly wages war on the human body. ... a stalemate. In pancreatic cancer, this stalemate—known as ... becoming aggressively malignant, a phenomena that is poorly ... in the laboratory of Salvatore Torquato, a Professor ... the conditions surrounding tumor dormancy and the switch ...
Breaking Biology News(10 mins):Improved electricity access has little impact on climate change 2Modeling tumor dormancy 2
... found a way of stimulating the immune systems ... the devastating plaques,that are characteristic of Alzheimer's disease.,For ... a,protein-based vaccine to slow the progression of the ... created a gene-based,vaccine aimed at stimulating the immune ...
... cells',responsible for hearing and balance. Traditionally viewed as ... for the normal development,of nerve cells responsible for ... research. The study is reported in the January ... scientists at the University,of Washington.,"Using zebrafish as a ...
... essential for 'hair cells',responsible for hearing and balance. ... may be essential for the normal development,of nerve ... new,University of Utah research. The study is reported ... is co-authored by scientists at the University,of Washington.,"Using ...
Cached Biology News:Gene vaccine for Alzheimer's disease shows promising results 2GenMAPP 2.0 released 2Harmful Bacterium Commonly Found in Poultry May Survive Refrigeration and Frozen Storage Combined 2
Mouse monoclonal antibody raised against a partial recombinant ZNF155. NCBI Entrez Gene ID = ZNF155...
... macroporous polystyrene beads for hydrophobic interaction chromatography. ... adsorbing organics of molecular weight less than ... useful for the adsorption of nonpolar substances ... removal of detergents such as Triton X-100 ...
... : Fusion protein corresponding to ... human Mi-2 Formulation: 0.7M Tris-glycine, ... sodium azide, containing 30% glycerol ... immunoblot on Hela nuclear extract ...
... Phospho-Specific(Thr 222 ) ... 10 mM sodium HEPES, 100 ... 7.5. Recognizes the ~47 kDa ... (MAPKAPK-2) protein phosphorylated at Thr ...
Biology Products: