Health & Medicine
ALS Latest News And Updates: Gene Study Showed Promise In Future ALS Therapies
Johnson D
First Posted: Oct 06, 2016 05:50 AM EDT
Scientists around the world have been pouring their efforts into finding a cure for amyotrophic lateral sclerosis, or ALS. Now, researchers from Ben-Gurion University of the Negev (BGU) say their new gene study could lead to the development of new treatments for this neurodegenerative disease.
As everybody may have already known ALS or what others may call as Lou Gehrig's disease is a fatal neurodegenerative disease that causes the death of motor neurons, which control voluntary muscles. This condition may cause progressive weakness and paralysis due to muscle atrophy which could lead to difficulty in speaking, swallowing and eventually breathing. The disease typically starts between ages 40 and 60, and the average survival from onset to death is two to five years.
According to Eurekalert, in 90 percent of the cases, the cause is unknown but, about 10 percent of the total cases were found to be genetically inherited. It was also found that about 20 percent of these genetic cases are caused by mutations in the SOD1 gene (superoxide dismutase), which usually leads to the accumulation of "misfolded" SOD1 proteins that trigger selective killing of motor neurons.
"Correct protein folding is critically important, which is why we are focusing on the diverse set of complex cellular mechanisms, including molecular chaperones, that promote efficient folding and prevent toxicity," says Dr. Adrian Israelson, who heads the Cellular and Molecular Neurodegeneration Lab in the BGU Department of Physiology and Cell Biology.
Recently, the study has reported for the first time that "endogenous multifunctional protein-macrophage migration inhibitory factor (MIF)," a gene known to regulate cell inflammation and immunity, acts as a chaperone for misfolded SOD1 in a mouse model. The researchers showed that completely eliminating MIF in a mutant SOD1 mouse model of familial ALS increased misfolded SOD1 accumulation. This also hastened the disease onset and late disease progression and shortened the lifespan of mice revealing mutant SOD1, reported Medical Xpress.
The researchers believe that their findings can set an example for ALS treatments in the future. UPI reported Dr. Israelson saying, "This study provides insight into the potential therapeutic role of MIF in suppressing the selective accumulation of misfolded SOD1 in ALS by modulating MIF levels." His laboratory focuses on cellular and molecular mechanisms that could cause the onset and progress of neurodegenerative diseases like Alzheimer's, Parkinson's and Huntington's diseases, with special emphasis on ALS.
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First Posted: Oct 06, 2016 05:50 AM EDT
Scientists around the world have been pouring their efforts into finding a cure for amyotrophic lateral sclerosis, or ALS. Now, researchers from Ben-Gurion University of the Negev (BGU) say their new gene study could lead to the development of new treatments for this neurodegenerative disease.
As everybody may have already known ALS or what others may call as Lou Gehrig's disease is a fatal neurodegenerative disease that causes the death of motor neurons, which control voluntary muscles. This condition may cause progressive weakness and paralysis due to muscle atrophy which could lead to difficulty in speaking, swallowing and eventually breathing. The disease typically starts between ages 40 and 60, and the average survival from onset to death is two to five years.
According to Eurekalert, in 90 percent of the cases, the cause is unknown but, about 10 percent of the total cases were found to be genetically inherited. It was also found that about 20 percent of these genetic cases are caused by mutations in the SOD1 gene (superoxide dismutase), which usually leads to the accumulation of "misfolded" SOD1 proteins that trigger selective killing of motor neurons.
"Correct protein folding is critically important, which is why we are focusing on the diverse set of complex cellular mechanisms, including molecular chaperones, that promote efficient folding and prevent toxicity," says Dr. Adrian Israelson, who heads the Cellular and Molecular Neurodegeneration Lab in the BGU Department of Physiology and Cell Biology.
Recently, the study has reported for the first time that "endogenous multifunctional protein-macrophage migration inhibitory factor (MIF)," a gene known to regulate cell inflammation and immunity, acts as a chaperone for misfolded SOD1 in a mouse model. The researchers showed that completely eliminating MIF in a mutant SOD1 mouse model of familial ALS increased misfolded SOD1 accumulation. This also hastened the disease onset and late disease progression and shortened the lifespan of mice revealing mutant SOD1, reported Medical Xpress.
The researchers believe that their findings can set an example for ALS treatments in the future. UPI reported Dr. Israelson saying, "This study provides insight into the potential therapeutic role of MIF in suppressing the selective accumulation of misfolded SOD1 in ALS by modulating MIF levels." His laboratory focuses on cellular and molecular mechanisms that could cause the onset and progress of neurodegenerative diseases like Alzheimer's, Parkinson's and Huntington's diseases, with special emphasis on ALS.
See Now: NASA's Juno Spacecraft's Rendezvous With Jupiter's Mammoth Cyclone