Amyotrophic Lateral Sclerosis (ALS) related Cell Products
Motor neurons are the main target of the debilitating neurodegenerative disease amyotrophic lateral sclerosis (ALS), which causes muscle weakening, atrophy, and ultimately paralysis. There is currently no cure, but studies have shown that in vitro cell models and cell therapies are promising new avenues for the treatment of ALS.
In vitro cell models play an important role in ALS research. These models are able to mimic the pathological features of ALS, such as decreased synaptic protein levels, neuromuscular disconnection, and reduced nerve endings. By improving the generation of hiPSC-derived motor neuron subtypes, the pathological mechanisms of ALS can be better studied and future treatments supported.
Our products include various cell types, such as cell lines, neural stem/progenitor cells and iPSC-derived cells, which can secrete a variety of nerve growth factors, enhance neuroprotection and prevent motor neuron death. In addition, our in vitro models can recapitulate the pathological features of ALS patients, such as decreased synaptic protein concentration and neuromuscular disconnection, providing important support for drug development and optimization of treatment strategies.
Please feel free to browse through our related products list to discover the ideal tools for your ALS research. For more information about our products or pricing, please contact us.
Explore ALS Models Based on Your Research Objective
SOD1 gene mutation is one of the major causes of familial ALS, accounting for approximately 18.9% of familial ALS and 1.2% of sporadic ALS. SOD1 is an antioxidant enzyme whose main function is to scavenge free radicals in cells and protect cells from oxidative damage. However, when SOD1 is mutated, its antioxidant function is weakened, leading to the accumulation of free radicals, which in turn causes mitochondrial dysfunction and neuronal damage.
Cat. No | Product Name |
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NCL-2105-P133-IX | iNeuTM Human Motor Neurons (SOD1 mutant, A4V, HOM) |
NRZP-0323-ZP42 | iNeuTM Human iPSC, ALS SOD1 H44R, Fibroblast-derived |
NRZP-0323-ZP56 | iNeuTM Human iPSC, ALS SOD1, PBMC-derived |
The relationship between the C9ORF72 gene and ALS is primarily reflected in the expansion of the hexanucleotide repeat sequence (GGGGCC) of the C9ORF72 gene, which is one of the major genetic causes of ALS and frontotemporal dementia (FTD). Expansion of this repeat sequence can lead to multiple pathogenic mechanisms, including RNA toxicity, loss of protein function, and nuclear transport disorders.
Cat. No | Product Name |
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NCL-2101-ZP35 | iNeuTM Human Motor Neuron Progenitors (C9ORF72 expansion) (Healthy) |
NCL-2101-ZP36 | iNeuTM Human Motor Neuron Progenitors- Amyotrophic Lateral Sclerosis (ALS) (C9ORF72 expansion) |
TDP-43 is a DNA and RNA binding protein involved in RNA processing and gene expression regulation. In ALS patients, TDP-43 often shows abnormal cytoplasmic distribution instead of its normal nuclear localization, and this mislocalization is closely associated with neurodegeneration. Abnormal aggregation and dysfunction of TDP-43 can lead to cytoskeletal dysfunction, disrupted axonal transport, and abnormal accumulation of intracellular vesicles.
Cat. No | Product Name |
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NCL-2105-P134-IX | iNeuTM Human Motor Neurons (TDP-43 mutation, M337V, HET) |
NCL-2105-P135-IX | iNeuTM Human Motor Neurons (TDP-43 mutation, Q331K, HET) |
NCL-2105-P136-IX | iNeuTM Human Motor Neurons (TDP-43 mutation, M337V, HOM) |
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