Research teams unite for research on Lou Gehrig's Disease

by Chelsea Whyte

Lisa Miller and Paul Gelfand, biophysical chemists at the U.S. Department of Energy's Brookhaven National Laboratory, recently visited the Advanced Photon Source at Argonne National Laboratory to supplement their research into the cause of amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease.

They're studying the genetic form of the disease, which is caused by a mutation in a gene that instructs cells to make a particular protein within the . When the gene is mutated, the protein folds abnormally, clumping into aggregates that cause paralysis and eventually death.

"This protein uses two metals to fold correctly – copper and zinc," says Gelfand. "We can use the x-ray beams at a synchrotron to look at the status of the copper and zinc relative to the motor neurons in the spinal cord."

Getting a clear picture of structural deformations in extremely small biological samples requires tiny x-ray beams—smaller than what's currently available at Brookhaven's National Synchrotron Light Source (NSLS).

"We're using the very tiny beams at the Advanced Photon Source in order to focus in on those tiny aggregates, to understand how much copper there is, how much zinc there is, if there's any, to help us understand why the aggregates form and why paralysis happens," says Miller.

"If we understand why the protein is misfolding, it's possible to create drugs or develop techniques to rescue the protein before it misfolds," Gelfand says.

This video is not supported by your browser at this time.

The scientists' work will continue at the National Synchrotron Light Source II at Brookhaven, using the nanoscale-size beams at the Submicron Resolution X-Ray Spectroscopy Beamline when it begins operation in 2015.

add to favorites email to friend print save as pdf

Related Stories

The role of metal ions in amyotrophic lateral sclerosis

Mar 10, 2011

(PhysOrg.com) -- Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, is a progressive neurodegenerative disease that affects motor neurons in the spinal cord, leading to muscle weakness, ...

Time-lapse movies from an infrared microscope

Nov 26, 2013

(Phys.org) —Infrared beams produced at facilities like the National Synchrotron Light Source represent the lower-energy part of the emitted light spectrum, yet are still much brighter than other sources, ...

High-speed X-ray 'camera' beamline taking shape at NSLS-II

Nov 21, 2013

(Phys.org) —"Phew!" Andrei Fluerasu breathes a sigh of relief as he looks over the plans for the beamline he has been building with a team of scientists, engineers and technicians at the National Synchrotron Li ...

Brain iron as an early predictor of Alzheimer's disease

Jun 17, 2011

Early and correct diagnosis of Alzheimer’s disease (AD) is important for reasons that go beyond treatment. These include more time to make critical life decisions, planning for future care, and maximizing ...

Recommended for you

New biomedical implants accelerate bone healing

2 hours ago

A major success in developing new biomedical implants with the ability to accelerate bone healing has been reported by a group of scientists from the Department of Restorative Dentistry, University of Malaya. ...

A new way to prevent the spread of devastating diseases

19 hours ago

For decades, researchers have tried to develop broadly effective vaccines to prevent the spread of illnesses such as HIV, malaria, and tuberculosis. While limited progress has been made along these lines, ...

User comments