Amyotrophic Lateral Sclerosis (ALS) Research Reagents

Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease characterized by progressive loss of upper and lower motor neurons. Key pathogenic mechanisms include TDP-43 proteinopathy (~97% of cases), SOD1 misfolding, C9orf72 repeat expansion, and FUS aggregation. The recent FDA approvals of tofersen (anti-SOD1 ASO) and emerging TDP-43-targeted therapies are driving demand for validated biomarker reagents.

Research Use Only (RUO)Not intended for diagnostic or therapeutic procedures.

Amyotrophic Lateral Sclerosis (ALS) Research Reagents - key pathogenic pathways and research targets

Fig. 1 Key pathogenic pathways and research targets. abinScience product targets highlighted in orange.

abinScience provides validated antibodies, recombinant proteins, and ELISA kits for key ALS/motor neuron disease research targets. All products are manufactured by our parent company AtaGenix Laboratories under ISO quality systems. Browse products below or contact us for custom antibody development.

Key Research Targets

TDP-43 (TARDBP) — Cytoplasmic TDP-43 mislocalization and aggregation is the hallmark pathology of ~97% of ALS cases. Anti-TDP-43 and anti-phospho-TDP-43 (pS409/410) antibodies enable pathological staging, CSF biomarker development, and aggregation mechanism studies.
→ Browse TDP-43 antibodies & proteins

SOD1 — Missense SOD1 mutations cause ~2% of ALS through toxic gain-of-function protein aggregation. Anti-SOD1 antibodies support misfolded SOD1 detection, tofersen (ASO) treatment response monitoring, and gene therapy research.
→ Browse SOD1 antibodies & proteins

Neurofilament Light Chain (NfL) — Serum/CSF NfL is the leading prognostic and pharmacodynamic biomarker in ALS clinical trials. Anti-NfL antibodies enable ultrasensitive immunoassay development for disease progression monitoring and treatment response assessment.

FUS — FUS mutations cause juvenile-onset ALS through RNA-binding protein mislocalization and stress granule dysfunction. Anti-FUS antibodies support nuclear/cytoplasmic localization studies and FUS-positive inclusion detection by IHC.

References

1. Feldman EL, et al. Amyotrophic lateral sclerosis. Lancet. 2022;400(10360):1363-1380. DOI

2. Miller TM, et al. Trial of antisense oligonucleotide tofersen for SOD1 ALS. N Engl J Med. 2022;387(12):1099-1110. DOI

3. Neumann M, et al. Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science. 2006;314(5796):130-133. DOI

4. Verde F, et al. Neurofilament light chain in serum for the diagnosis of amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry. 2019;90(2):157-164. DOI