Lou Gehrig's Disease or Amyotrophic Lateral Sclerosis (ALS) is a neurological disorder characterized by progressive degeneration of motor neuron cells in the spinal cord and brain, which ultimately results in paralysis and death. The disease takes its less-scientific name from Lou Gehrig, a baseball player with the New York Yankees in the late 1920s and 1930s, who was forced to retire in 1939 as a result of the loss of motor control caused by the disease.
In 1991, a team of researchers linked familial ALS to chromosome 21. Two years later, the SOD1 gene was identified as being associated with many cases of familial ALS. The enzyme coded for by SOD1 carries out a very important function in cells: it removes dangerous superoxide radicals by converting them into non-harmful substances. Defects in the action of this enzyme mean that the superoxide radicals attack cells from the inside, causing their death. Several different mutations in this enzyme all result in ALS, making the exact molecular cause of the disease difficult to ascertain.
Recent research has suggested that treatment with drugs called antioxidants may benefit ALS patients. However, since the molecular genetics of the disease are still unclear, a significant amount of research is still required to design other promising treatments for ALS.
While much about Alzheimer's disease remains a mystery, scientists do know that part of the disease's progression involves a normal protein called tau, aggregating to form ropelike inclusions within brain cells that eventually strangle the neurons.
Up to 90 percent of people with amyotrophic lateral sclerosis (ALS) report that they have no family history of the disease.
The most frequent genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD)- rare and related neurological disorders marked by progressive deterioration of motor or cognitive abilities- may be due to errors in RNA splicing, an intermediary step for translating genetic instructions into functional proteins.
A new study finds that a decades-old drug used to treat malaria lowers levels of a biomarker linked to the inherited form of amyotrophic lateral sclerosis, also known as ALS and Lou Gehrig's Disease.
Ben-Gurion University of the Negev researchers are developing a new therapy for Amyotrophic Lateral Sclerosis using part of an existing FDA-approved drug that restores the central nervous system's (CNS) immune defenses and increases life expectancy.
For the first time, a variant in UBQLN4 gene has been associated with Lou Gehrig's disease or amyotrophic lateral sclerosis (ALS) - a progressive disease resulting in the loss of nerve cells that control muscle movement, which eventually leads to paralysis and death.
The U.S. Food and Drug Administration today approved Radicava (edaravone) to treat patients with amyotrophic lateral sclerosis, commonly referred to as Lou Gehrig's disease.
Scientists report a significant step toward combatting two degenerative brain diseases that chip away at an individual's ability to move, and think.
A research team led by St. Jude Children's Research Hospital immunologists has discovered how a set of proteins delays the "executioner" machinery that kills damaged or infected cells in a process called necroptosis.
A study by Indiana University researchers has identified 24 compounds -- including caffeine -- with the potential to boost an enzyme in the brain shown to protect against dementia.
Working with yeast and human cells, researchers at Johns Hopkins say they have discovered an unexpected route for cells to eliminate protein clumps that may sometimes be the molecular equivalent of throwing too much or the wrong trash into the garbage disposal.
In cells, DNA is first converted to RNA, and RNA is next converted to proteins--a complicated process involving several other steps. Nonsense-mediated RNA decay is a processing pathway in cells that, like a broom, cleans up erroneous RNA to prevent its productive conversion into an aberrant protein, which could lead to disease.
A computer interface that can decipher the thoughts of people who are unable to communicate could revolutionize the lives of those living with completely locked-in syndrome, according to a new paper publishing January 31st, 2017 in PLOS Biology.
A bacterial by-product known to be important in maintaining gut health may slow the progression of amyotrophic lateral sclerosis, or ALS - a progressive, neurodegenerative disease.
Under ordinary circumstances, the protein tau contributes to the normal, healthy functioning of brain neurons. In some people, though, it collects into toxic tangles that damage brain cells.
Illumina, Inc. and Bio-Rad Laboratories, Inc. today announced the launch of the Illumina® Bio-Rad® Single-Cell Sequencing Solution at the J.P. Morgan Healthcare Conference. The comprehensive solution is the first next-generation sequencing (NGS) workflow for single-cell analysis, providing researchers the ability to investigate the coordinated contribution of individual cells in tissue function, disease progression, and therapeutic response.
The Orphan Disease Center in the Perelman School of Medicine at the University of Pennsylvania has established a new Program of Excellence for Motor Neuron Disease.
A tool that uses light to manipulate matter inside living cells has begun to explain how proteins assemble into different liquid and gel-like solid states, a key to understanding many critical cellular operations.
When you're suddenly able to understand someone despite their thick accent, or finally make out the lyrics of a song, your brain appears to be re-tuning to recognize speech that was previously incomprehensible.
Researchers at the Virginia Tech Carilion Research Institute have identified a naturally occurring molecule that has the potential for preserving sites of communication between nerves and muscles in amyotrophic lateral sclerosis (ALS) and over the course of aging -- as well as a molecule that interferes with this helpful process.