Amyotrophic Lateral Sclerosis: overview, antibodies, and disease-related markers

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• What is Amyotrophic lateral sclerosis?
• Download our Interactive ALS pathway
• References

What is Amyotrophic lateral sclerosis?     

​​​​​​​​Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND) or Lou Gehrig’s disease, is an adult-onset, progressive, neurological disease that results in the destruction of neurons controling voluntary muscles, namely those in the motor cortex, brainstem, and spinal cord.

The pathological hallmark of ALS is the presence of inclusion bodies (abnormal aggregations of protein) in the cytoplasm of motor neurons. In the vast majority of cases, the main component of the inclusion bodies is TDP-43 protein, however, in those with SOD1 or FUS mutations, the main component is SOD1 protein or FUS protein, respectively^1,2.

Genetic studies have implicated deficits in autophagy and/or mitophagy in the onset of the disease³.

Amyotrophic lateral sclerosis –​ interactive pathway 

Study key genes and molecular mechanisms involved in the development of ALS with our interactive poster. It also helps you easily find the best products for your target of interest by providing pop ups and product tables that include Abcam's full product offering including cell lines, antibodies, proteins, ELISA, Multiplex, biochemicals, conjugates and more.

Click on the preview image below to download our interactive pathway.

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References

1. Saberi, S., Stauffer, J.E., Schulte, D.J., Ravits, J. Neuropathology of amyotrophic lateral sclerosis and its variants. Neurol Clin. 33, 855–876 (2015).
2. Blokhuis, A.M., Groen, E.J., Koppers, M., van den Berg, L.H., Pasterkamp, R.J. Protein aggregation in amyotrophic lateral sclerosis. Acta Neuropathol. 125, 777–794 (2013).
3. Evans, C.S., Holzbaur, E.L.F. Autophagy and mitophagy in ALS. Neurobiol. Dis. 122, 35–40 (2019).  ​