ALS High-Throughput Phenotypic Screening Assay Service
Amyotrophic lateral sclerosis (ALS) remains a devastating enigma. Its intricate pathogenesis, still not yet fully understood, defies conventional drug development approaches. Traditional methods, tethered to pre-defined targets, often fall short in capturing the disease's multifaceted nature. Our ALS high-throughput phenotypic screening service shatters these limitations. We leverage a powerful, target-agnostic strategy, allowing you to explore the vast landscape of cellular responses to potential therapeutics. By observing how compounds influence complex ALS-relevant phenotypes, we unlock novel targets and mechanisms of action previously hidden from view. For further information regarding the products and services provided, project-specific consultation, and pricing, please submit an inquiry here.
ALS Patient-Specific Cell Models
- iPSC-Derived Motor Neurons
We utilize induced pluripotent stem cells (iPSCs) derived directly from patients carrying well-characterized ALS-associated gene mutations, including SOD1, C9orf72, and FUS. These iPSCs are differentiated into motor neurons, providing a physiologically relevant platform to model familial ALS pathogenesis. This model is ideal for high-throughput drug screening, enabling the identification of compounds that specifically target genetic subtypes of ALS.
Fig.1 Representative images of iPSC-derived motor neurons.1, 3
- Sporadic ALS Fibroblasts
We employ fibroblasts from patients with sporadic ALS, those without known genetic mutations, to capture the broader spectrum of the disease. Utilizing high-content imaging with markers such as EEA1 and FUS, we perform detailed phenotypic characterization of these cells. This allows us to identify subpopulations exhibiting dysregulation of key pathways, such as the FUS-ALS pathway, providing critical insights into the underlying disease mechanisms.
Advanced Imaging and Automation Assays
- Live-Cell Dynamic Imaging
We employ fluorescent probes to capture real-time neuronal calcium signaling, enabling the visualization and quantification of critical ALS phenotypes, such as excitotoxicity. This dynamic imaging approach provides a window into the functional changes occurring in ALS neurons, revealing subtle yet crucial alterations.
- Multi-Dimensional Phenotypic Analysis
Our high-content imaging (HCI) platform allows for the simultaneous monitoring of multiple phenotypic parameters, including cell morphology, activity, and secretion. This multi-parametric analysis unveils the intricate complexity of ALS excitatory phenotypes, providing a comprehensive understanding of disease mechanisms. This system allows for the finding of compounds that can correct multiple disease parameters at once. This comprehensive view enables the identification of compounds that effectively address the multifaceted nature of ALS.
Case Study: Human iPSC-derived motor neurons exhibit electrophysiological functions, and ALS motor neurons demonstrate hyperexcitability.
Fig.2 Evaluation of ALS neuronal activity and overstimulation using iPSC-derived motor neurons.2, 3
Our comprehensive ALS high-throughput phenotypic screening service integrates state-of-the-art technology with the expertise of our dedicated scientific team, propelling your drug development efforts forward. We offer a powerful combination of precise cellular phenotyping and multi-dimensional analysis, delivering efficient and reliable screening solutions tailored to your research needs. Please contact us for customized solutions!
References
- Phatnani, Hemali, et al. "An integrated multi-omic analysis of iPSC-derived motor neurons from C9ORF72 ALS patients." Iscience 24.11 (2021).
- Kondo, Tosho, et al. "Rapid and robust multi-phenotypic assay system for ALS using human iPS cells with mutations in causative genes." International journal of molecular sciences 24.8 (2023): 6987.
- Distributed under Open Access license CC BY 4.0, without modification.

- Mouse Anti-SCN5A Monoclonal Antibody (CBP708) (Cat#: NAB-0720-Z2720)
- iNeuMab™ Anti-F-Spondin/SPON1 Antibody, Clone 3F4 (Cat#: NRZP-0822-ZP4740)
- iNeuMab™ Mouse Anti-EFNB2 Monoclonal Antibody (CBP1159) (Cat#: NAB-0720-Z4396)
- Mouse Anti-Human α-Synuclein Phospho (Tyr39) (CBP3706) (Cat#: NAB201250LS)
- iNeuMab™ Rabbit Anti-Alpha-synuclein (CBP1631) (Cat#: NAB-08-PZ079)
- iNeuMab™ Mouse Anti-SHANK3 Monoclonal Antibody (CBP929) (Cat#: NAB-0720-Z3477)
- iNeuMab™ Rabbit Anti-LRRK2 Monoclonal Antibody (CBP1887) (Cat#: NAB-08-PZ735)
- iNeuMab™ Mouse Anti-LRP1 Monoclonal Antibody (CBP3363) (Cat#: NAB-0720-Z6479)
- Mouse Microglia Cell Line BV-2, Immortalized (Cat#: NCL2110P153)
- iNeu™ Human Sensory Neurons (Cat#: NCL-2103-P62)
- iNeu™ Human Schwann Cell (Cat#: NCL-2103-P63)
- Mouse Microglia N9 (Cat#: NCL2110P073)
- Rat Schwann Cells RSC96, Immortalized (Cat#: NCL-2108P21)
- Rat Olfactory Ensheathing Cells (Cat#: NRZP-1122-ZP162)
- Mouse Retinal Ganglion Cell Line RGC-5 (Cat#: NCL2110P154)
- Human Dental Pulp Stem Cells (Cat#: NRZP-1122-ZP113)
- Mouse Retinal Ganglion Cells (Cat#: NCL2110P145)
- iNeu™ Human Motor Neurons (Cat#: NCL-2103-P71)
- Human Tau Aggregation Kit (Cat#: NRP-0322-P2173)
- Amyloid beta 1-42 Kit (Cat#: NRP-0322-P2170)
- Human Poly ADP ribose polymerase,PARP Assay Kit (Cat#: NRZP-1122-ZP62)
- Beta Amyloid (1-42), Aggregation Kit (Cat#: NRZP-0323-ZP200)
- Beta Amyloid (1-40), Aggregation Kit (Cat#: NRZP-0323-ZP199)
- Human GFAP ELISA Kit [Colorimetric] (Cat#: NPP2011ZP383)
- Alpha Synuclein Aggregation Kit (Cat#: NRZP-1122-ZP15)
- Alpha-Synuclein Aggregation Assay Kit (Cat#: NRZP-1122-ZP37)
- VSV-eGFP (Cat#: NTA-2011-ZP20)
- Dextran, NHS Activated (Cat#: NRZP-0722-ZP124)
- AAV2 Full Capsids, Reference Standards (Cat#: NTC2101070CR)
- Mouse Parkinson disease (autosomal recessive, early onset) 7 (Park7) (NM_020569) clone, Untagged (Cat#: NEP-0621-R0133)
- Tau Antisense Oligonucleotide (IONIS-MAPTRx) (Cat#: NV-2106-P29)
- Human apolipoprotein E (APOE) (NM_000041) ORF clone, Untagged (Cat#: NEP-0421-R0232)
- App Rat amyloid beta (A4) precursor protein (App)(NM_019288) ORF clone, Untagged (Cat#: NEP-0421-R0053)
- Human superoxide dismutase 3, extracellular (SOD3) (NM_003102) ORF clone, Untagged (Cat#: NEP-0521-R0808)
- Human huntingtin (HTT) (NM_002111) ORF clone, Myc-DDK Tagged (Cat#: NEP-0521-R0497)
- Human presenilin 1 (PSEN1), transcript variant 2 (NM_007318) ORF clone, TurboGFP Tagged (Cat#: NEP-0421-R0140)
- Mouse SOD1 shRNA Silencing Adenovirus (Cat#: NV-2106-P14)
- Lenti of Mouse synuclein, alpha (Snca) transcript variant (NM_001042451) ORF clone, mGFP Tagged (Cat#: NEP-0521-R0864)
- ABCA1 Antisense Oligonucleotide (NV-2106-P27) (Cat#: NV-2106-P27)
- NeuroBiologics™ Mouse Cerebrospinal Fluid (Cat#: NRZP-0822-ZP497)
- NeuroBiologics™ Human Cerebrospinal Fluid (Cat#: NRZP-0822-ZP491)
- NeuroBiologics™ Pig Cerebrospinal Fluid (Cat#: NRZP-0822-ZP498)
- NeuroBiologics™ Rat Cerebrospinal Fluid (Cat#: NRZP-0822-ZP496)
- NeuroBiologics™ Monkey Cerebrospinal Fluid (Cat#: NRZP-0822-ZP495)
- NeuroPro™ Anti-idursulfase BBB Shuttle Protein (Cat#: NRZP-0423-ZP497)
- NeuroPro™ Anti-PON1 BBB Shuttle Protein (Cat#: NRZP-0423-ZP507)
- NeuroPro™ Anti-NAGLU BBB Shuttle Protein (Cat#: NRZP-0423-ZP506)
- NeuroPro™ Anti-IDS BBB Shuttle Protein (Cat#: NRZP-0423-ZP503)
- NeuroPro™ Anti-EPO BBB Shuttle Protein (Cat#: NRZP-0423-ZP508)
- NeuroPro™ Anti-Erythropoietin BBB Shuttle Protein (Cat#: NRZP-0423-ZP499)
- NeuroPro™ Anti-GDNF BBB Shuttle Protein (Cat#: NRZP-0423-ZP500)
- NeuroPro™ Anti-SGSH BBB Shuttle Protein (Cat#: NRZP-0423-ZP505)
- NeuroPro™ Anti-GDNF BBB Shuttle Protein (Cat#: NRZP-0423-ZP509)
- NeuroPro™ Anti-TNFR BBB Shuttle Protein (Cat#: NRZP-0423-ZP501)