Tools for amyotrophic lateral sclerosis research
Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease, is a neurogenerative disease characterized by the loss of upper and lower motor neurons. While over 40 genes associated with ALS have been identified, only 10-15% of ALS cases are familial; the remaining 85-90% of cases are classified as sporadic ALS.
Whilst there is significant heterogeneity in the pathogenesis of ALS, there are common pathways being explored to help with the identification of new drug targets and biomarkers. These include impaired proteostasis, autophagy dysregulation, glutamate excitotoxicity and neuroinflammation.
Abcam’s broad catalog of industry-leading neuroscience reagents includes antibodies to relevant targets (such as TDP-43, C9orf27, FUS and SOD1), as well as iPSC-derived disease models as vital pre-clinical models. Explore our range to find the best tools to move your experiments forward.
Impaired proteasome and autophagy dysregulation antibodies
Advances in large-scale genomic analyses and novel pre-clinical models have uncovered a variety of causative genes and risk factors for ALS, many of which are implicated in proteostasis and the regulation of autophagy.
Abcam’s portfolio of gold-standard reagents includes recombinant antibodies raised against targets implicated in ALS—including TDP-43, SOD1, C9orf72, and FUS genes— allowing you to uncover novel mechanisms of pathogenesis. Our antibodies are broadly validated across applications (including IHC) and species using brain tissue samples (including patient samples where relevant) to ensure specificity in your experiments.
Product highlights
Anti-TDP43 antibody [EPR5810]
- Validated in ALS patient samples
- Broadly validated across relevant applications including WB, ICC/IF, flow cytometry, IHC-P and IHC-Fr
- Knock-out validated
Anti-Superoxide Dismutase 1 antibody [EP1727Y]
- Cited in over 40 publications
- Broadly validated across relevant applications including WB, ICC/IF, flow cytometry and IHC-P in mouse, rat and human samples
- Knock-out validated
Anti-C9orf72 antibody [EPR22021]
- C9orf72 quantification validated by Western blot
- Tested in human brain cell lines and brain tissue
Glutamate excitotoxicity and oxidative stress antibodies
A major pathophysiological mechanism in ALS involves the excessive stimulation of postsynaptic glutamate receptors. Excitotoxicity caused by excessive neuronal firing contributes to oxidative stress and injury of motor neurons.
Better understanding the susceptibility of neurons to excitotoxic injury in ALS patients is an important avenue for new therapeutics. Abcam has the antibodies you need to study glutamatergic neurons including common markers to glutamate receptors (eg GRIN1/NMDAR1) and glutamate transporters (eg vGlut1 and EAAT1).
Product highlights:
Anti-NMDAR1 antibody [EPR2481(2)]
- Knock-out validated
- Validated for WB and ICC/IF in mouse, human and rat samples
- Cited in over 60 publications
Anti-Glutamate Receptor 1 (AMPA subtype) antibody [EPR19522]
- Broadly validated across IP, WB, IHC-Fr, IHC-P;
- Reacts with mouse, rat and human samples
- Cited in over 20 publications
Anti-VGluT1 antibody [EPR22269]
- Broadly validated across ICC/IF, IP, WB, IHC-Fr, IHC-P
- Reacts with mouse, rat and human samples
- Optimized for IHC using the Leica BONDTM system
Neuroinflammation antibodies and kits
Neuroinflammation is a pathological hallmark of many neurodegenerative diseases including ALS. Whilst pre-clinical models have shown that both astrocytes and microglia are likely to contribute to neurotoxicity in ALS, the precise mechanisms remain largely unknown.
Abcam offers the some of the most highly cited recombinant antibodies for microglia and astrocyte markers including GFAP, Iba1 and TMEM119. These are broadly validated across applications, including in many cases for IHC using human and mouse brain tissue.
Product highlights:
Anti-GFAP antibody [EPR1034Y]
- Broad coverage of clone – H/M/R reactivity for mIHC, flow cytometry, IHC-P, IHC-Fr, ICC/IF, IP and WB
- Validated with human cerebellum tissue
- 14 five-star reviews
Anti-Iba1 antibody [EPR16588] - Goat IgG (Chimeric)
Goat recombinant monoclonal antibody
Anti-TMEM119 antibody [28-3] - Microglial marker
- The most-cited TMEM119 antibody available with 190 citations
- Knock-out validated
iPSC-derived models for ALS
We’re proud to partner with leading experts in induced pluripotent stem cell (iPSC) technologies including bit.bio and BrainXell, who are advancing the development of new models for complex diseases. Our iPSC-derived neuronal cell lines can be used as vital pre-clinical models for studying the molecular mechanisms of ALS and to screen potential novel therapeutic interventions.
Product highlights:
ioGlutamatergic Neurons TDP-43 M337V homozygous - Human iPSC derived cells
- A powerful next generation model to study ALS and FTD in research and drug discovery
- Engineered from human iPSCs using opti-ox™, a precise reprogramming technology
- Cells express pan-neuronal and glutamatergic markers TUBB3, MAP2 and VGLUT2 by day 11
Spinal Motor Neuron - Human iPSC derived cells (Male, WC-30)
- High neuronal purity (>80%) comprising over 70% motor neurons
- For even greater physiological relevance, co-culture protocols are available for use with spinal astrocytes
- A GFP-expressing alternative to this product is also available
