HOME >> BIOLOGY >> NEWS
Mitochondria in spinal cords is ALS target according to UCSD medical researchers

The selective killing of spinal cord neurons in amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, occurs when tiny cellular components called mitochondria actively recruit a mutant disease-causing protein into specific neuron cells, according to new research by University of California, San Diego (UCSD) School of Medicine investigators.

Published in the July 8, 2004 issue of the journal Neuron, the findings identify mitochondria as the focus of ALS toxicity and provide the first explanation of how a mutant protein called SOD1 that occurs in all cells in the body is damaging only to specific neuron cells. The result is ALS, a progressive degeneration of motor nerve cells in the spinal cord that leads to wasted muscles and premature death in middle-aged adults.

Found in all cells, mitochondria provide cellular energy in their role as the body's power generators. In addition, mitochondria are intricately involved in a process called apoptosis, or programmed cell death, which is the body's normal method of disposing of damaged, unwanted or unneeded cells.

"We believe that when the mutant SOD1 binds to mitochondria, it affects the ability of these components to generate cell energy," said the study's senior author, Don Cleveland, Ph.D., a UCSD professor of medicine, neurosciences, and cellular and molecular medicine, and a faculty member of the Ludwig Institute for Cancer Research.

He added that "the SOD1 may also tip the balance and induce mitochondria to send a cell death signal to the motor neurons."

In laboratory studies with tissue from animals and humans, the UCSD team determined that the mutant SOD1 ignores all other mitochondria in cells and tissue outside of the spinal cord, and binds only to the mitochondria in the large motor neurons that begin in the spinal cord and extend down the arms and legs. They found SOD1 both inside the mitochondria and coated on the mitochondria's outer components.
'"/>

Contact: Sue Pondrom
spondrom@ucsd.edu
619-543-6163
University of California - San Diego
7-Jul-2004


Page: 1 2

Related biology news :

1. Mitochondrial genes cause nuclear mischief
2. Milestone In Mitochondrial Structure Revealed
3. Nerve cells guided to repair spinal damage: Technique
4. Yale researchers receive $4.5 million grant to study spinal cord repair
5. Study of ProCord for neurologically complete spinal cord injury
6. Combination therapy dramatically improves function after spinal cord injury in rats
7. Naropin now approved for intrathecal (spinal) administration in the European Union (EU)
8. Chemical gradient steers nerve growth in spinal cord
9. Dying nerves cause even more harm after spinal cord injury
10. Gene-expression atlas will provide new direction for brain and spinal-cord studies
11. New gene for rare inherited paralysis may aid other spinal cord research, too

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


(Date:6/19/2020)... ... ... Join experts from Envigo Lauren Klaskala, Project Manager – Custom Transgenic ... Wednesday, July 8, 2020 at 11am EDT (4pm BST/UK). , Nuclease-based technologies such as ... modifying the genome may be more straightforward, the challenge to create an optimal model ...
(Date:6/11/2020)... , ... June 09, 2020 , ... Superior Controls, ... industrial automation and IT solutions for the life sciences industry, is pleased to announce ... Development working out of the company’s Seabrook, New Hampshire office. In his new ...
(Date:6/5/2020)... ... ... Greffex, a pioneering vaccine and gene therapy company based in Houston, Texas, welcomes ... a global perspective to make an impact in a post pandemic world,” said Co-Chairman ... independent, director of the company. , “The Greffex board is excited to have Neil ...
Breaking Biology News(10 mins):
(Date:6/28/2020)... ... ... The Indoor Lab, a leading provider of sensor solutions for monitoring foot ... ensure cleanliness, distancing and occupancy with its patent pending WipeAway™ technology using Artificial Intelligence ... a health performance index system that scores all aspects of compliance similar to the ...
(Date:6/23/2020)... ... June 23, 2020 , ... Kerafast Inc. , developers ... announced the availability of the Delta-G-VSV Pseudotyping System for coronavirus research applications. The ... of SARS-CoV-2 viral entry and COVID-19 vaccine effectiveness at just biosafety level 2 ...
(Date:6/23/2020)... ... June 23, 2020 , ... Nobilis ... announced that it has filed an Investigational New Drug application (IND) with the ... of its lead drug candidate NBTX-001 in patients with panic disorder (PD). , ...
(Date:6/11/2020)... ... June 09, 2020 , ... TRX Systems, developer of ... Solution, a highly accurate, contact tracing solution for identification of employees who ... a core disease control measure, is a key strategy for preventing further spread ...
Breaking Biology Technology:
Cached News: