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  

1

GOOD

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:5/23/2013)... Utah (PRWEB) May 23, 2013 Today, ... employer mandate . Zane Benefits, which provides comprehensive ... is the leader in defined contribution plans ... care reform, a common question from employees is “does ... Zane Benefits’ website, not necessarily. The health care reform ...
(Date:5/23/2013)... (PRWEB) May 23, 2013 Onlinefitnessreview.com recently ... Blast Diet to determine what makes this diet work ... published review highlights three main components of the program: ... a positive mindset and the personal motivation that is ... every 7 days to keep the metabolism rate in ...
(Date:5/23/2013)... City, UT (PRWEB) May 23, 2013 ... , a comprehensive radiology learning environment for all ... prebuilt lessons and assessments, transforms the learning experience ... radiology curriculum. , “Until RADPrimer 3.0, ... second to none,” comments Dr. B.J. Manaster, Professor ...
(Date:5/23/2013)... modified version of the polio virus might one ... Scientists at Duke Cancer Institute said the ... engineered form of the virus that is harmless ... therapy shows promise in the treatment of glioblastoma, ... said. "These early results are intriguing," principal ...
(Date:5/23/2013)... Plano, TX (PRWEB) May 23, 2013 ... the Marketplace , Chaplains have been a part of ... this Memorial Day, like those preceding it, these men ... service to the United States of America. As ... trend of their migration to the workplace chaplaincy world. ...
Breaking Medicine News(10 mins):Health News:Zane Benefits Publishes New Information on the Employer Mandate 2Health News:Zane Benefits Publishes New Information on the Employer Mandate 3Health News:Onlinefitnessreview.com Publishes 7 Day Belly Blast Diet Review 2Health News:RADPrimer 3.0 Introduces Comprehensive Radiology Training Curriculum 2Health News:Modified Polio Virus May Help Fight Brain Tumors, Study Suggests 2Health News:Memorial Day Focus Spotlight on Military Chaplains Transitioning in the Marketplace 2Health News:Memorial Day Focus Spotlight on Military Chaplains Transitioning in the Marketplace 3
... technology called the Cone beam computerized tomography (CBCT) is ... complicated oral problems.// ,J. Martin Palomo and ... Case Western Reserve University School of Dental Medicine and ... dental health and biological sciences at the University of ...
... two protein complexes which is found to protect cell ... number of chromosomes leading to cell instability which leads ... research team from Mayo Clinic have found that the ... which maintains the euploidy state in the cell and ...
... Ontario’s hospitals are getting longer, according to Government data which ... the Conservative health critic.// The waiting time rose from 34 ... cancer surgery, while it rose from 139 days to 142 ... days for cardiac surgeries, while in the case of angioplasty, ...
... reported to have been cracked by the researchers from ... treatment of the ailment which causes severe diarrhea,// abdominal ... Dasval. The strain has caused infections to breakout in ... now commence for the other antibiotic resistance genes and ...
... Research Institute (SERI) found that increasing the contrast of ... to the visually impaired. // ,The U.S. study ... to help millions suffering eye diseases. People who may ... macular degeneration, diabetic retinopathy and other causes of low ...
... president of Sandia National Laboratories, Gerry Yonas was the first to ... ,This led to the development of the BiNational Sustainability Laboratory (BNSL), ... Teresa. This will mainly augment the border security force and ... Mexico. ,The monetary inputs for the lab ...
Cached Medicine News:Health News:New Computerized Imaging Systems Help In Better Dental Treatment Planning 2Health News:Visually impaired prefer specially enhanced TV 2Health News:Visually impaired prefer specially enhanced TV 3
(Date:5/23/2013)... , May 23, 2013 Hearing health affects a ... younger, and remain socially and professionally engaged, he should address ... overriding message that the Better Hearing Institute (BHI) ... National Men,s Health Week , which leads up to ... runs from June 10 through June 16. ...
(Date:5/23/2013)... , May 23, 2013  A recently published ... Houston, Texas , demonstrates that 3D ... increasing cancer detection. The study, "Implementation ... Observational Study," was led by Stephen L. ... issue of the American Journal of Roentgenology ...
(Date:5/23/2013)... May 23, 2013  Hadasit Bio-Holdings Ltd. (TASE: HDST, ... companies, based on intellectual property developed and owned ... ,s foremost medical research center, today announced that ... significant funding from the Israeli Ministry of Industry ... to support product development and advance clinical trials. ...
Breaking Medicine Technology:Men Who Want to Stay Active, Feel Younger, and Remain Socially and Professionally Engaged Should Address Hearing Loss, BHI Advises 2Men Who Want to Stay Active, Feel Younger, and Remain Socially and Professionally Engaged Should Address Hearing Loss, BHI Advises 3Men Who Want to Stay Active, Feel Younger, and Remain Socially and Professionally Engaged Should Address Hearing Loss, BHI Advises 4First Large-Scale U.S. Study Validates the Benefits of 3D Mammography (Breast Tomosynthesis) Exams 2First Large-Scale U.S. Study Validates the Benefits of 3D Mammography (Breast Tomosynthesis) Exams 3Hadasit Bio-Holdings Ltd. Portfolio Company - Cell Cure Neurosciences Ltd. Received Additional Government Support 2
... , CAMBRIDGE, Mass., Nov. 19 Ligon Discovery, ... find drugs against high-value targets, today announced that it ... Ventures. The company will use the capital to complete ... high-value disease protein targets. With this investment, incTANK Ventures ...
... , Part of ... Md., Nov. 19 The U.S. Food and Drug ... the International Internet Week of Action (IIWA), intended to ... http://www.newscom.com/cgi-bin/prnh/20090824/FDALOGO ) , During the effort, the FDA,s Office ...
Cached Medicine Technology:Cambridge-based Ligon Discovery Raises $1M in Seed Funding 2Cambridge-based Ligon Discovery Raises $1M in Seed Funding 3UPDATE: FDA Issues 22 Warning Letters to Web site Operators 2
... for the quantitative determination of ... kinetic, lag phase of less ... linear phase of at least ... Linearity: 100 mg/dL (35.6 mmol/L ...
For the quantitative determination of direct bilirubin in human serum. Linearity: 20 mg/dl...
... the first microplate spectrophotometer designed specifically to ... per week. With rugged hardware, proven optical ... the barrier of traditional spectrophotometry to obtain ... can easily read up to eight, 1 ...
The PerkinElmer LS 45 Luminescence spectrometer offers versatility, reliability and ease-of-use for routine analyses in an affordable package....
Medicine Products: