Amyotrophic Lateral Sclerosis: overview, antibodies, and disease-related markers
Related
Explore ALS and access the tools you need to support your research.
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 which control 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, respectively1,2.
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, which will help you find the best tools for your research in ALS.
Click on the preview image below to download our interactive pathway.
Recombinant monoclonal antibodies and proteins for ALS disease research
Get reproducible, batch-to-batch consistency using recombinant monoclonal antibodies and proteins for your ALS research. These are some of our best-selling products.
Antibodies
| Gene | Target | abID | Application | Species |
| C9ORF72 | C9ORF72 | ab221137 | WB | Mouse, Rat, Human |
| FUS | TLS/FUS | ab124923 | WB, IHC-P, Flow Cyt, ICC/IF | Mouse, Rat, Human |
| SOD1 | Superoxide dismutase | ab51254 | WB, Flow Cyt, IHC-P, ICC/IF | Rat, Human |
| VCP | VCP | ab109240 | WB, IP, IHC-P, Flow Cyt, ICC/IF | Mouse, Rat, Human |
| TARDBP | TDP43 | ab109535 | ICC/IF, Flow Cyt, IHC-Fr, WB, IHC-P | Mouse, Rat, Human |
Proteins
Target | Species | Expression system | abID |
Superoxide Dismutase 1 | Human | Escherichia coli | ab84642 |
VCP | Human | Baculovirus infected Sf9 cells | ab269130 |
TDP43 | Human | Escherichia coli | ab269030 |
Disease-associated markers
To help you get ahead in your ALS research, we’ve compiled the best markers to use in conjunction with our disease-specific antibodies.
| Gene | Target | abID | Application | Species |
| ISL1 | Islet 1 | ab109517 | IHC-Fr, ICC/IF, WB, IP, Flow Cyt | Mouse, Chicken, Human |
| VIM | Vimentin | ab92547 | ICC/IF, WB, Flow Cyt, IHC-P, IHC-Fr | Mouse, Rat, Human, Rhesus monkey |
Other key cell and neuronal markers from Abcam
Check out our range of neuronal markers for supporting your research.
| Neural markers | Marker group | Recommended product |
| Ki67 | Proliferating cell marker | ab16667 |
| GFAP | Schwann cell/radial glia/astrocyte | ab33922, ab68428 |
| VIM | Radial glia | ab92547 |
| NeuN | Mature neuron | ab177487 |
| MAP2 | Mature neuron | ab183830 |
| TUBB3 | Immature neuron | ab78078 |
| MBP | Oligodendrocyte | ab218011 |
| PSD95 | Mature neuron | ab18258 |
| NES | Radial glia | ab105389 |
| DCX | Immature neuron | ab18723 |
| SYP | Mature neuron | ab32127 |
| NOTCH1 | Astrocyte | ab52627 |
| TH | Dopaminergic neuron | ab137869 |
| S100B | Astrocyte | ab52642 |
| MAPT | Mature neuron/axons | ab80579, ab32057, ab109390 (phospho S396) |
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).