Researchers at Northwestern University say they have discovered a common cause behind the mysterious and deadly affliction of amyotrophic lateral sclerosis, or Lou Gehrig's disease, that could open the door to an effective treatment. In 1941, New York Yankee baseball superstar Lou Gehrig died at 37 of the disease that now carries his name.
A variety of proteins perform different functions within cells, and Deng and Chen led research that discovered a key protein, ubiquilin2, in the ALS mystery.
Ubiquilin2 in spinal and brain system cells is supposed to repair or dispose of other proteins as they become damaged. The researchers discovered a breakdown of this function in ALS patients.
When Ubiquilin2 is unable to remove or repair damaged proteins, the damaged proteins begin to pile up in the cells, eventually blocking normal transmission of brain signals in the spinal cord and brain, leading to paralysis.
There are three forms of ALS: "familial," which is hereditary and passed through genes; nonhereditary, which is called "sporadic"; and ALS that targets the brain, called "ALS/dementia."
src
(2021) - https://www.chicagotribune.com/lifestyles/health/ct-met-northwestern-als-breakthrough-20110822-story.html
(2005) - https://alsnewstoday.com/news-posts/2015/11/05/ubiquilin-2-may-future-target-als-treatment/
ref - Ubiquilin 2: a component of the ubiquitin-proteasome system with an emerging role in neurodegeneration
#als
A variety of proteins perform different functions within cells, and Deng and Chen led research that discovered a key protein, ubiquilin2, in the ALS mystery.
Ubiquilin2 in spinal and brain system cells is supposed to repair or dispose of other proteins as they become damaged. The researchers discovered a breakdown of this function in ALS patients.
When Ubiquilin2 is unable to remove or repair damaged proteins, the damaged proteins begin to pile up in the cells, eventually blocking normal transmission of brain signals in the spinal cord and brain, leading to paralysis.
There are three forms of ALS: "familial," which is hereditary and passed through genes; nonhereditary, which is called "sporadic"; and ALS that targets the brain, called "ALS/dementia."
src
(2021) - https://www.chicagotribune.com/lifestyles/health/ct-met-northwestern-als-breakthrough-20110822-story.html
(2005) - https://alsnewstoday.com/news-posts/2015/11/05/ubiquilin-2-may-future-target-als-treatment/
ref - Ubiquilin 2: a component of the ubiquitin-proteasome system with an emerging role in neurodegeneration
#als