Thursday, January 28, 2016

Hope for ALS patients

Posted By on Thu, Jan 28, 2016 at 2:27 PM

click to enlarge Steve Gleason has worked hard to make people aware of ALS and to raise money for research. - CHERYL GERBER
  • Cheryl Gerber
  • Steve Gleason has worked hard to make people aware of ALS and to raise money for research.
Researchers have found a naturally occurring protein that could open a pathway for treating amyotrophic lateral sclerosis (ALS).

A research paper written by J. Gavin Daigle, a doctoral candidate at the Louisiana State University Health New Orleans School of Graduate Studies, was published this month in the online journal Acta  Neurpathologica.

Pur-alpha, a protein that is present in neuron cells, could hold the key to slowing neurodegeneration in patients with ALS, the paper concluded.
ALS disease that affects nerve cells in the brain and spinal cord that signal and control muscles. When those neuron cells die, the brain no longer can communicate or control muscles. Patients lose their ability to move, speak and sometimes swallow or breathe. There is no cure. 

click to enlarge ALS ASSOCIATION
  • ALS Association
Daigle and researchers from LSU and universities in Pittsburgh and Philadelphia, Pennsylvania, Norfolk, Virginia, and Bethesda, Maryland, used neuronal cell lines from ALS Patients, which all have gene mutations, and examined the mechanisms that cause neurodegeneration. They found the degeneration was triggered when cells produced “stress granules” to protect them against damage from heat, disease and other stresses — a normal process — but were unable to break down and expel the granules as healthy cells do once the trauma ended, causing the cells to die. Increasing Pur-alpha in such cells helps with both actions, the paper said.

“[Pur-alpha] definitely needs to be tested for it’s therapeutic potential,” Daigle said in a phone interview. “Currently we don’t have any drugs or compounds that could target [Pur-alpha to make it produce more]. That will be the next stage.” Researchers also are conducting tests to determine whether Pur-alpha itself can be delivered as a supplement much as insulin is, he said.

Mutations in several genes, including FUS and DNA- and RNA-binding proteins, have been associated with ALS, but not all patients are born with mutations, Daigle says.
“For 20 percent to 25 percent of ALS patients, the gene mutation is hereditary,” he said. “The remaining 75 to 80 percent of patients are what we call ‘sporadic’ because there is no detectable or known genetic cause.”

The study was published shortly before Gleason, a documentary about former New Orleans Saints defensive back Steve Gleason, was screened to tears and cheers at the Sundance Film Festival. Gleason was diagnosed with ALS several years ago at the age of 34 and has worked diligently to raise awareness of the disease and raise funds for research.
Daigle and his team did not specifically study the effect concussions or prolonged mental and physical stress have on ALS, but he says other researchers are making a connection.

“Research is emerging that environmental factors such as stress — [whether] concussion, traumatic brain injury or post-traumatic stress — can influence the behavior of your neurology and neurophysiology,” Daigle says. “There have been studies that have shown that people who return from war are more susceptible to neurodegenerative conditions. … There is most likely an association and a gene environment interaction, but we don’t know if it’s causal or not.” The paper pointed out that other researchers have also linked Pur-alpha deficiency to brain development disorders.

Daigle says many ALS patients don’t develop symptoms until later in life, and the national ALS Association says the disease generally strikes people between 40 and 70 years old.

“As you age, many of the processes become less efficient,” Daigle says. “We’re modeling an aging disease in a petri dish (using cell lines from ALS patients), looking specifically at the neurons that are associated with the disease. This really brings up a good tool, a good model for studying drug compounds and a potential Pur-alpha supplement to see if we’re able to alter this and enhance the cell’s ability to deal with the stress and protect it from dying.”

The breakthrough could help with other neurodegenerative diseases, such as Parkinson’s and Alzheimer’s disease, although the disease progression and parts of the brain affected can be different, he said.
“This could definitely extend to other neurodegenerative conditions,” Daigle said. “A lot of these are the same mechanisms that exist in all neurodegenerative conditions. … A lot of these processes we’re looking at could have implications for all neurodegenerative conditions.”

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