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ALS is a motor neuron disease, which is a group of neurological disorders that selectively affect motor neurons, the cells that control voluntary muscles of the body. [3] Other motor neuron diseases include primary lateral sclerosis (PLS), progressive muscular atrophy (PMA), progressive bulbar palsy , pseudobulbar palsy , and monomelic ...
A score of 0 on a question would indicate no function while a score of 4 would indicate full function. [4] [5] This scale has been useful for doctors in diagnosing patients, measuring disease progression and also for researchers when selecting patients for a study and measuring the potential effects of a clinical trial. [4] [6]
In the United States and Canada, the term motor neuron disease usually refers to the group of disorders while amyotrophic lateral sclerosis is frequently called Lou Gehrig's disease. [ 2 ] [ 5 ] [ 22 ] In the United Kingdom and Australia, the term motor neuron(e) disease is used for amyotrophic lateral sclerosis, [ 3 ] [ 4 ] although is not ...
[4] [5] ALS has an oligogenic mode of inheritance, meaning that mutations in two or more genes are required to cause disease. [6] C9orf72 is the most common gene associated with ALS, causing 40% of familial cases of ALS, as well as a small percentage of sporadic cases; [7] it also causes about 25% of familial cases of frontotemporal dementia. [6]
Actor David Niven began showing symptoms of ALS in interviews, and would be officially diagnosed in 1980. The 11th century monk Hermann of Reichenau had a lifelong disease that is considered likely to have been ALS. This would make him one of the earliest known patients of the disease. Chairman Mao Zedong was reported to have been suffering ...
PMA affects only the lower motor neurons, in contrast to amyotrophic lateral sclerosis (ALS), the most common MND, which affects both the upper and lower motor neurons, or primary lateral sclerosis, another MND, which affects only the upper motor neurons. The distinction is important because PMA is associated with a better prognosis than ALS.
Also, some studied have introduced the transgenic strain of C. elegans, which has a mutation in a gene related to ALS for example, and crossed them with the transgenic nlp-29 GFP reporter strain, resulting in fluorescent markers to the cells that are expressing these mutated genes, which can be used to monitor the disease development and ...
The ALS Therapy Development Institute (ALS TDI) is a non-profit biotechnology research organization focused on finding treatments for amyotrophic lateral sclerosis (ALS). With a staff including more than 30 scientists, it operates a research and development program centered on ALS.