HOME >> BIOLOGY >> NEWS
Tracing a killer's path in Lou Gehrig's disease

Two papers in the July 8, 2004, issue of Neuron shed light on how a mutant form of a normally protective protein in the cells of sufferers of amyotrophic lateral sclerosis targets the "power plants" of motor neurons, killing the neurons and ultimately resulting in paralysis and death.

The culprit is a mutant form of the enzyme superoxide dismutase (SOD1), which normally protects cells from harmful reactive oxygen species. However, about 3% of people with ALS have a mutant form of the enzyme.

Don Cleveland and his colleagues conducted experiments in mice showing that the mutant SOD1 protein selectively migrates to the mitochondria of spinal cord neurons, where it links itself to the mitochondrial membrane.

Mitochondria are the cells' power plants, converting glucose into chemical energy that drives cellular processes. They are also the headquarters of the cells' "suicide" machinery, which triggers the death of cells that are malfunctioning or no longer needed--a process called apoptosis.

"This study offers one of the first glimpses into how cells from the spinal cord may be selectively targeted for degeneration in models of ALS," wrote the researchers.

"We propose that the universal association of SOD1 mutant exclusively within affected tissues represents the common property of these mutants that initiates a cascade of damage," they wrote.

In a second paper, Robert H. Brown, Jr., and his colleagues shed light on a specific mechanism by which the mutant SOD1 kills spinal cord neurons once it reaches their mitochondria.

Brown and his colleagues discovered that, in both mouse and human spinal cord neurons, mutant SOD1 specifically links itself to a key mitochondrial protein, called Bcl-2, that normally suppresses the apoptotic machinery of cells. Bcl-2 is also important in maintaining the function of the mitochondrial membrane. The researchers did not find evidence of such linkage in liver cell mitochondria,
'"/>

Contact: Heidi Hardman
hhardman@cell.com
617-397-2879
Cell Press
7-Jul-2004


Page: 1 2

Related biology news :

1. Tracing genes, biologists show lizard migration is traced to Florida
2. Tracing the neural circuitry of second sight
3. Tracing the evolution of sex chromosomes
4. Elusive cancer killers deep-sea hideout discovered after a nearly 20-year hunt
5. First of crop killers genome sequence available
6. New findings are a window into the late stages of Lou Gehrigs disease
7. Abnormally high number of Lou Gehrigs disease cases identified among Gulf War veterans
8. Gene therapy delays death in mouse with symptoms of Lou Gehrigs disease
9. Salk news: gene therapy for Lou Gehrigs disease
10. Gene discovery may shed light on carpel tunnel syndrome and Lou Gehrigs disease
11. Gene therapy holds promise for treating inherited Lou Gehrigs Disease

Post Your Comments:
*Name:
*Comment:
*Email:


(Date:6/14/2017)... (NYSE: IBM ) is introducing several innovative partner startups ... collaboration between startups and global businesses, taking place in ... nine startups will showcase the solutions they have built with ... France is one of the ... percent increase in the number of startups created between 2012 ...
(Date:5/16/2017)... 16, 2017   Bridge Patient Portal , ... MD EMR Systems , an electronic medical ... GE, have established a partnership to build an ... the GE Centricity™ products, including Centricity Practice Solution ... These new integrations will allow healthcare delivery ...
(Date:4/19/2017)... New York , April 19, 2017 ... competitive, as its vendor landscape is marked by the ... the market is however held by five major players ... Safran. Together these companies accounted for nearly 61% of ... of the leading companies in the global military biometrics ...
Breaking Biology News(10 mins):
(Date:10/12/2017)... ... October 12, 2017 , ... ... platform specifically designed for life science researchers to analyze and interpret datasets, ... Franklin, who made a major contribution to the discovery of the double-helix ...
(Date:10/11/2017)... ... ... Personal eye wash is a basic first aid supply for any work environment, but most ... you rinse first if a dangerous substance enters both eyes? It’s one less decision, and ... unique dual eye piece. , “Whether its dirt and debris, or an acid or alkali, ...
(Date:10/11/2017)... , Oct. 11, 2017  VMS BioMarketing, a leading ... a nationwide oncology Clinical Nurse Educator (CNE) network, which will ... need for communication among health care professionals to enhance the ... nurses, office staff, and other health care professionals to help ... breast cancer. ...
(Date:10/11/2017)... ... October 11, 2017 , ... ... implantation and pregnancy rates in frozen and fresh in vitro fertilization (IVF) ... and maternal age to IVF success. , After comparing the results from the ...
Breaking Biology Technology:
Cached News: