Navigation Links
Malfunctioning gene associated with Lou Gehrig's disease leads to nerve-cell death in mice
Date:1/5/2011

cells. Expression of either human TDP-43 led to neuron loss in vulnerable forebrain regions; degeneration of part of the spinal cord tract; and muscle spasms in the mice. These effects recapitulate key aspects of FTLD and a subtype of ALS known as primary lateral sclerosis.

The JCI study showed that a dramatic loss of function causes nerve-cell death because normal mouse TDP-43 is eliminated when human mutated TDP-43 genes are put into the mice. Since cells regulate the exact amount of TDP-43, over-expression of the human TDP-43 protein prevents the mouse TDP-43 from functioning normally. Lee and colleagues think this effect leads to neuron death rather than clumps of TDP-43 because these clumps were rare in the mouse cells observed in this study. Lee says that it is not yet clear why clumps were rare in this mouse model when they are so prevalent in human post-mortem brain tissue of ALS and FTLD patients.

Neurodegeneration in the mouse neurons expressing TDP-43 -- both the normal and mutated human versions -- was accompanied by a dramatic downregulation of the TDP-43 protein mice are born with. What's more, mice expressing the mutated human TDP-43 exhibited profound changes in gene expression in neurons of the brain's cortex.

The findings suggest that disturbing the normal TDP-43 in the cell nucleus results in loss of normal TDP-43 function and gene regulatory pathways, culminating in degeneration of affected neurons.

Next steps, say the researchers, will be to look for the specific genes that are regulated by TDP-43 and how mRNA splicing is involved so that the abnormal regulation of these genes can be corrected.

At the same time, notes Lee, "We soon will launch studies of novel strategies to prevent TDP-43-mediated nervous system degeneration using this mouse model of ALS and FTLD."


'/>"/>

Contact: Karen Kreeger
karen.kreeger@uphs.upenn.edu
215-349-5658
University of Pennsylvania School of Medicine
Source:Eurekalert  

Page: 1 2

Related medicine news :

1. Certain genetic profiles associated with recurrence-free survival for non-small cell lung cancer
2. Certain pain medications do not appear to be associated with skin cancer risk
3. News brief: ALDH associated with worse overall survival in patients with pancreatic adenocarcinoma
4. New research shows emotional impact of low sexual desire and associated distress
5. Increased HAART coverage associated with 50 percent drop
6. Pretreatment SUV associated with head and neck cancer treatment outcomes, may help decide treatment plans
7. New developments in nanotechnology tackle the 2 biggest problems associated with chemotherapy
8. MedQuist Launches EHR Integration for Dictations Associated with Template-based Clinical Documentation
9. Hormone thought to slow aging associated with increased risk of cancer death
10. Common osteoporosis drugs are associated with a decrease in risk of breast cancer
11. Coffee Drinking and Caffeine Associated With Reduced Risk of Hospitalization for Heart Rhythm Disturbances
Post Your Comments:
*Name:
*Comment:
*Email:
Related Image:
Malfunctioning gene associated with Lou Gehrig's disease leads to nerve-cell death in mice
(Date:10/31/2014)... This Method to get a ... for practitioners. , The Hypopresive (aka Hipopressive) ... and Movement specializing in physical rehabilitation. Dr. Marcel ... reeducation that traditional abdominal exercises after pregnancy increased ... etc. , The Hipopressives exercises pull back in ...
(Date:10/31/2014)... 31, 2014 (HealthDay News) -- Surgery for low back ... in the United States you live, a new report ... low back pain at some point in their lives, ... medical care for a spine problem," co-author Brook Martin, ... Practice, said in a college news release. In ...
(Date:10/31/2014)... say that either they or a family member have ... latest issue of The Gerontological Society of America,s ... which goes on to show that Americans strongly support ... end-of-life health care wishes. , This PP&AR , ... features 12 articles that present new ways of understanding ...
(Date:10/31/2014)... 2014 Urban Hydroponics, Inc. (OTCQB:URHY), a ... it has signed a Binding Letter of Intent (LOI) ... cultivation sector - Urban Cultivator Inc. (“Urban Cultivator”), BC ... Metal Inc. (“W3 Metal” and together with Urban Cultivator ... to the LOI and an earlier non-binding term sheet, ...
(Date:10/31/2014)... October 31, 2014 Triple Board Certified ... hair restoration services to transform the appearance of military ... American Academy of Facial Plastic and Reconstruction Surgery (AAFPRS) ... to restore the facial hair Jones lost during an ... The pro-bono hair restoration procedure, valued around $16,000, took ...
Breaking Medicine News(10 mins):Health News:Super Models and European Celebrities’ Secret Flat Tummy System Comes to America 2Health News:Super Models and European Celebrities’ Secret Flat Tummy System Comes to America 3Health News:Spinal Surgery Varies by Region in U.S.: Study 2Health News:Advance directives can benefit patients, families, and health care system 2Health News:Urban Hydroponics Inc. Signs Definitive Agreement to Merge with Canadian Hydroponics Companies 2Health News:Urban Hydroponics Inc. Signs Definitive Agreement to Merge with Canadian Hydroponics Companies 3Health News:Urban Hydroponics Inc. Signs Definitive Agreement to Merge with Canadian Hydroponics Companies 4Health News:Urban Hydroponics Inc. Signs Definitive Agreement to Merge with Canadian Hydroponics Companies 5Health News:Miami Hair Restoration Specialist Transforms Wounded Soldier 2
... ... tracking, performance analysis, sales and management training, announced that its non-profit organization, PrideAmerica is ... Super Bowl Hearing Mission on Wednesday, February 3rd, from noon – 4 pm at ... ...
... NASHVILLE, Tenn. , Jan. 29 HCA ... quarter and fiscal year ended December 31, 2009 . ... Company,s year-end financial and accounting procedures. , (Logo: ... these uncertain economic times," said Richard M. Bracken , ...
... , DETROIT , Jan. 29 ... M.D., Ph.D., has been named the new president and chief ... thoracic oncologist, Dr. Bepler will begin his new position ... , In addition to his chief administrative duties, Dr. ...
... , CHICAGO , Jan. 29 ... decision, four national organizations representing dentists, physicians and veterinarians ... exclude health professionals from controversial new regulation intended to ... A letter sent to FTC Chairman Jon Leibowitz ...
... facial recognition systems, study authors suggest , FRIDAY, Jan. 29 ... as long as they think the faces are odd-shaped flowers, ... researchers from Australia and France determined that bees could be ... a sweet treat for doing so. , However, this doesn,t ...
... counterfeit Viagra, study says , FRIDAY, Jan. 29 (HealthDay ... especially drugs purporting to treat erectile dysfunction, are playing Russian ... best the drugs won,t help you and at worst they ... be wasting your money or you may actually be hurting ...
Cached Medicine News:Health News:CallSource joins Starkey Hearing Foundation to Tackle Children's Hearing Loss in Super Bowl Mission 2Health News:HCA Previews Fourth Quarter and Year-End 2009 Results 2Health News:HCA Previews Fourth Quarter and Year-End 2009 Results 3Health News:HCA Previews Fourth Quarter and Year-End 2009 Results 4Health News:HCA Previews Fourth Quarter and Year-End 2009 Results 5Health News:HCA Previews Fourth Quarter and Year-End 2009 Results 6Health News:HCA Previews Fourth Quarter and Year-End 2009 Results 7Health News:HCA Previews Fourth Quarter and Year-End 2009 Results 8Health News:Karmanos Cancer Institute in Detroit Names Gerold Bepler, M.D., Ph.D., as CEO 2Health News:Karmanos Cancer Institute in Detroit Names Gerold Bepler, M.D., Ph.D., as CEO 3Health News:Karmanos Cancer Institute in Detroit Names Gerold Bepler, M.D., Ph.D., as CEO 4Health News:Health Professionals Petition FTC For Relief From Red Flags Rule 2Health News:Health Professionals Petition FTC For Relief From Red Flags Rule 3Health News:Fake Drugs Bought on the Web Pose Big Health Risks 2Health News:Fake Drugs Bought on the Web Pose Big Health Risks 3
(Date:10/31/2014)... BARCELONA , October 31, 2014 ... to infringe two Australian patents of B. Braun Melsungen AG ... Australia safety IV catheters manufactured by Poly ... of Australia proceeding number VID 463 ... In the detailed judgment, Multigate was found to ...
(Date:10/31/2014)... , October 30, 2014 ... announced the addition of the  "Process Validation in ... their offering.      (Logo: http://photos.prnewswire.com/prnh/20130307/600769) , ... and Risk Based Approach to Process Validation for ... Management FDA Process Validation Guidance ...
(Date:10/31/2014)... , Oct. 31, 2014 Though therapies ... technologies are expected to show an annual increase of ... value of nearly $1 billion, according to Kalorama Information,s ... from proprietary stem cell therapeutics that have gained or ... "As drug developers tackle ever more complicated ...
Breaking Medicine Technology:B. Braun Wins Patent Disputes Against Poly Medicure Ltd's Australian Distributor Multigate Medical Devices Pty. Ltd. and Spanish Distributor Dextromédica S. L. 2Process Validation in the US and EU (Workshop, London) 2Process Validation in the US and EU (Workshop, London) 3Process Validation in the US and EU (Workshop, London) 4Report: Stem Cell Technologies Market Growing 2
... May 11, 2012  Amgen (NASDAQ: AMGN ) ... vice president of Research and Development at Amgen will ... Administration (FDA) stakeholder hearing on biosimilars. Miletich will urge members ... for biosimilars to establish approval standards that advance patient ...
... Mylan Inc. (Nasdaq: MYL ) today ... mg, 20 mg, 40 mg and 80 mg in ... has received marketing authorization from each country,s respective health ... is the generic version of Pfizer,s Lipitor® Tablets. This ...
Cached Medicine Technology:Amgen To Provide Testimony At FDA Stakeholder Hearing On Biosimilars 2Amgen To Provide Testimony At FDA Stakeholder Hearing On Biosimilars 3Amgen To Provide Testimony At FDA Stakeholder Hearing On Biosimilars 4Amgen To Provide Testimony At FDA Stakeholder Hearing On Biosimilars 5Amgen To Provide Testimony At FDA Stakeholder Hearing On Biosimilars 6Mylan Launches Generic Lipitor® in Five European Countries 2
... Laparoscopic Instruments incorporate a layered design unique ... and monitored to prevent stray electrosurgical burns ... by insulation failure or capacitive coupling; a ... built into all AEM Instruments provides a ...
... Instruments incorporate a layered design unique in ... monitored to prevent stray electrosurgical burns along ... insulation failure or capacitive coupling; a well-documented ... into all AEM Instruments provides a neutral ...
... 5mm AEM Laparoscopic Instruments incorporate a layered ... are shielded and monitored to prevent stray ... instrument caused by insulation failure or capacitive ... protective shield built into all AEM Instruments ...
... 5mm AEM Laparoscopic Instruments incorporate a layered ... are shielded and monitored to prevent stray ... instrument caused by insulation failure or capacitive ... protective shield built into all AEM Instruments ...
Medicine Products: