2. Disease Areas
  3. Neurological, Eye or Ear Disease
  4. Neurodegenerative Disease
  5. Amyotrophic Lateral Sclerosis

Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by the degeneration and death of both upper and lower motor neurons in the brain, brainstem, and spinal cord, leading to muscle weakness, atrophy, fasciculations, spasticity, and ultimately fatal paralysis. The disease typically manifests in middle adulthood, with about 10% of cases being familial and linked to genetic mutations such as those in the SOD1 gene. ALS exhibits clinical and genetic heterogeneity, with subtypes defined by inheritance patterns, age of onset, and associated conditions like frontotemporal dementia, though no distinct "type 1" classification is recognized in current literature. Pathological features include neuronal loss, intracellular protein aggregates, and specific inclusions in surviving motor neurons. The etiology is multifactorial, involving complex interactions between genetic susceptibility and environmental factors.

Amyotrophic Lateral Sclerosis (28):

Cat. No. Product Name CAS No. Purity Chemical Structure
  • HY-132580
    Tofersen 2088232-70-4
    Tofersen (BIIB067) is an antisense oligonucleotide and SOD1 mRNA inhibitor with an IC50 of 320 pM. Tofersen mediates RNase H-dependent degradation of SOD1 mRNA to reduce SOD1 protein levels in cerebrospinal fluid and serum. Tofersen downregulates cerebrospinal fluid neurofilament light chain, neurofilament heavy chain, amyloid-beta 1-40, amyloid-beta 1-42, neuropeptide Y, ubiquitin C-terminal hydrolase L1, neuropentraxins 1, 2, R, corticotropin-releasing hormone, IL-15, and serum neurofilament light chain, neurofilament heavy chain. Tofersen can be used for the research of superoxide dismutase 1-associated amyotrophic lateral sclerosis.
    Tofersen
  • HY-N12060
    Ginkgo biloba extract 90045-36-6
    Ginkgo biloba extract is a natural product that can be isolated from Ginkgo biloba leaves. Ginkgo biloba extract alleviates oxidative stress-induced neuronal apoptosis (Apoptosis) by stabilizing mitochondrial function, regulating Bcl-2 family proteins and inhibiting caspase activation. Ginkgo biloba extract alleviates testicular injury by upregulating SKP2 and inhibiting Beclin1-independent autophagy (Autophagy). Ginkgo biloba extract alleviates various types of neuronal damage in animal models. Ginkgo biloba extract reduces behavioral sensitization in rats. Ginkgo biloba extract counteracts Aβ-induced neurotoxicity by blocking a series of Aβ-triggered events, including glucose uptake, ROS accumulation, AKT activation, mitochondrial dysfunction, JNK and ERK 1/2 pathways, and apoptosis, and also interferes with the formation of Aβ oligomers. Ginkgo biloba extract is applicable to research related to cerebral hypoperfusion, testicular injury, Alzheimer's disease, Parkinson's disease, multi-infarct dementia, stroke, traumatic brain injury and amyotrophic lateral sclerosis.
    Ginkgo biloba extract
  • HY-153898
    rTRD01 1332175-56-0 99.10%
    rTRD01 is an orally active, specific TDP-43 binder that targets the RRM1 and RRM2 domains of TDP-43. rTRD01 partially disrupts the interaction between TDP-43 and c9orf72 repeat RNA, but does not affect the binding of TDP-43 to canonical binding sequences. rTRD01 exhibits significant neuroprotective effects in zebrafish models, improves motor function and protects against paraquat (a widely used herbicide)-induced neurodegeneration, with no teratogenicity at high concentrations. rTRD01 is not a general antioxidant and cannot counteract Rotenone (HY-B1756)-induced neuronal death. rTRD01 can be used to study amyotrophic lateral sclerosis and other TDP-43 proteinopathies.
    rTRD01
  • HY-P99213
    Ozanezumab 1310680-64-8 99%
    Ozanezumab (GSK1223249) is a humanized, Fc-inactivated monoclonal antibody that targets the nervous system protein Nogo-A. Ozanezumab promotes neurite outgrowth and axonal regeneration by neutralizing Nogo-A signaling. Ozanezumab is used for research on neurodegenerative diseases such as amyotrophic lateral sclerosis and multiple sclerosis[1].
    Ozanezumab
  • HY-14604
    Xaliproden hydrochloride 90494-79-4 98.40%
    Xaliproden (SR57746) hydrochloride (SR57746A) is an orally active, highly selective 5-HT1A receptor agonist. Xaliproden hydrochloride activates pertussis toxin-sensitive G protein-coupled signaling cascades, as well as the PKC, ERK1/ERK2, Akt and p21 Ras/MEK-1 pathways. Xaliproden hydrochloride also downregulates the JNK/p66/c-Jun signaling pathway, induces phosphorylation of the shc adaptor protein, regulates extracellular dopamine and 5-HT levels, and induces [35S]GTPγS labeling in rat brain structures rich in 5-HT1A receptors. Xaliproden hydrochloride exerts neurotrophic, neuroprotective, renoprotective, anti-inflammatory, anti-apoptotic, anti-fibrotic and analgesic effects. Xaliproden hydrochloride also enhances NGF-induced neurite outgrowth, promotes motor neuron survival, attenuates renal tubular injury and inhibits chemotherapy-induced mechanical allodynia, without activating or altering NGF-induced TrkA receptor activation. Xaliproden hydrochloride can be used in the research of motor neuron disease, diabetic nephropathy, chemotherapy-induced peripheral neuropathy, amyotrophic lateral sclerosis, Alzheimer's disease, acute tonic nociceptive pain, inflammatory pain, depression and anxiety.
    Xaliproden hydrochloride
  • HY-181846
    nTRD22 1065567-62-5
    nTRD22 is an RNA-binding allosteric modulator targeting TDP-43. nTRD22 binds to the N-terminal domain of TDP-43, thereby allosterically regulating the RNA-binding domain of TDP-43 and reducing its RNA-binding ability. nTRD22 decreases the TDP-43 protein level in primary motor neurons. nTRD22 alleviates motor dysfunction in amyotrophic lateral sclerosis models. nTRD22 is applicable to related research on amyotrophic lateral sclerosis.
    nTRD22
  • HY-186072
    NT-0527 2771019-10-2 99.80%
    NT-0527 is a selective, orally active, and brain-permeable NLRP3 inflammasome inhibitor. NT-0527 can specifically block the formation of the NLRP3 inflammasome, resulting in the reduction in the maturation and release of IL-1β, exhibit inhibition on CYP2C19. NT-0527 displays anti-inflammatory activity in the mouse LPS (HY-D1056) /ATP (HY-B2176)-induced peritonitis model. NT-0527 can be used for the research of neuroinflammatory disorders (Parkinson's disease, Alzheimer's disease, amyotrophic lateral sclerosis) and peripheral inflammatory disorders (type II diabetes, atherosclerosis, gout, etc.) associated with NLRP3 inflammasome.
    NT-0527
  • HY-181406
    eIF2B activator-1 3118633-46-5
    eIF2B activator-1 (Compound 7a) is a eIF2B activator with a pEC50 value of 7.3. eIF2B activator-1 exhibits significant hERG inhibitory activity, with a pIC50 value of 5.7. eIF2B activator-1 can be used in research related to Huntington's disease, Parkinson's disease, and amyotrophic lateral sclerosis (ALS).
    eIF2B activator-1
  • HY-18102
    GLPG0492 1215085-92-9 99.85%
    GLPG0492 is an orally active, non-steroidal selective androgen receptor modulator. GLPG0492 exerts functional transactivation by binding to the ligand-binding domain of the receptor, exhibiting preferential partial agonist activity in muscle and bone tissues with low activity in reproductive tissues. GLPG0492 effectively counteracts muscle atrophy-related pathways, significantly enhances muscle strength, maintains motor ability, reduces fibrosis and improves electrophysiological parameters. GLPG0492 prevents immobilization-induced muscle atrophy and regulates muscle mass homeostasis, serving as a valuable tool compound for studies on Duchenne muscular dystrophy, muscle loss and various types of disuse musculoskeletal atrophy.
    GLPG0492
  • HY-148629
    GDC-0134 1637394-01-4 99.18%
    GDC-0134 (RG6000) is a modulator targeting dual leucine zipper kinase (DLK) that can cross the blood-brain barrier. By inhibiting the kinase activity of DLK, GDC-0134 blocks the activation of the downstream JNK signaling pathway, suppresses DLK-dependent retrograde signal transduction of axon-to-soma degeneration, and exerts neuroprotective activity. GDC-0134 reduces TDP-43 protein aggregation and decreases the degree of neuromuscular junction denervation in motor neurons. GDC-0134 can be used in the research of amyotrophic lateral sclerosis (ALS), Alzheimer's disease and other DLK-related neurodegenerative diseases.
    GDC-0134
  • HY-153399
    Fosigotifator 2415715-84-1
    Fosigotifator (ABBV-CLS-7262) is a brain penetrant, orally active EIF2b (eukaryotic initiation factor) activator. Fosigotifator stabilizes the elF2B complex and boosts the activity, including that of complexes carrying pathogenic vanishing white matter (VWM) mutations. Fosigotifator is an integrated stress response inhibitor (ISRI). Fosigotifator can be used for amyotrophic lateral sclerosis research.
    Fosigotifator
  • HY-153399A
    Fosigotifator THAM sodium 2945073-88-9 98.03%
    Fosigotifator (ABBV-CLS-7262) THAM sodium is a brain penetrant, orally active EIF2b (eukaryotic initiation factor) activator. Fosigotifator THAM sodium stabilizes the elF2B complex and boosts the activity, including that of complexes carrying pathogenic vanishing white matter (VWM) mutations. Fosigotifator THAM sodium is an integrated stress response inhibitor (ISRI). Fosigotifator THAM sodium can be used for amyotrophic lateral sclerosis research.
    Fosigotifator THAM sodium
  • HY-128128
    ASN04421891 570365-12-7 99.00%
    ASN04421891 is a GPR17 agonist with nanomolar EC50 and high specificity. ASN04421891 promotes oligodendrocyte precursor cell maturation to mature myelinating oligodendrocytes. ASN04421891 can be used for the research of cerebral ischaemia, cardiac ischaemia, renal ischaemia, cerebral trauma, multiple sclerosis, schizophrenia, depression, alzheimer's disease, alzheimer-like dementia, parkinson's disease, huntington's chorea, amyotrophic lateral sclerosis (ALS), neuroinflammatory disorders.
    ASN04421891
  • HY-W611371
    FP802 61694-81-3
    FP802 is an orally active potent TwinF interface inhibitor that disrupts and detoxifies the NMDAR/TRPM4 death complex. FP802 exerts powerful neuroprotective effects in the 5xFAD mouse model of Alzheimer’s disease (AD) by preventing cognitive decline, preserving neuronal structural integrity, reducing amyloid-β plaque formation, and mitigating mitochondrial pathology. FP802 stops loss of motor neurons, reduces serum neurofilament light chain (NfL) levels, improves motor performance, and extends life in a mouse model of amyotrophic lateral sclerosis (ALS). FP802 can be used for AD and ALS research.
    FP802
  • HY-W611371A
    FP802 dihydrochloride 2490401-57-3 99.84%
    FP802 dihydrochloride is an orally active potent TwinF interface inhibitor that disrupts and detoxifies the NMDAR/TRPM4 death complex. FP802 dihydrochloride exerts powerful neuroprotective effects in the 5xFAD mouse model of Alzheimer’s disease (AD) by preventing cognitive decline, preserving neuronal structural integrity, reducing amyloid-β plaque formation, and mitigating mitochondrial pathology. FP802 dihydrochloride stops loss of motor neurons, reduces serum neurofilament light chain (NfL) levels, improves motor performance, and extends life in a mouse model of amyotrophic lateral sclerosis (ALS). FP802 dihydrochloride can be used for AD and ALS research.
    FP802 dihydrochloride
  • HY-132580C
    Tofersen scrambled control
    Tofersen scrambled control is a scrambled control antisense oligonucleotide of Tofersen (HY-132580) with a scrambled sequence in the target binding region.
    Tofersen scrambled control
  • HY-N15352
    C20 Sphingomyelin (d18:1/20:1) 222403-67-0
    C20 Sphingomyelin (d18:1/20:1) is a sphingomyelin substance. The level of C20 Sphingomyelin (d18:1/20:1) is associated with amyotrophic lateral sclerosis and primary lateral sclerosis.
    C20 Sphingomyelin (d18:1/20:1)
  • HY-P992436
    PAS-003
    PAS-003 is a humanized monoclonal antibody targerting α5β1 integrin. PAS-003 modulates immune cell migration, immune cell adhesion, improves behavior, and improves survival in ALS mouse models. PAS-003 can be used for the research of amyotrophic lateral sclerosis.
    PAS-003
  • HY-156600A
    Bevemipretide trihydrochloride 2589640-11-7
    Bevemipretide trihydrochloride (SBT-272 trihydrochloride) is a blood-brain barrier-permeable mitochondrial function repair agent. Bevemipretide trihydrochloride stabilizes cardiolipin in the inner mitochondrial membrane, restores mitochondrial structure, respiratory function, motor capacity and upper motor neuron health. Bevemipretide trihydrochloride reduces astrogliosis and microgliosis in mice with amyotrophic lateral sclerosis. Bevemipretide trihydrochloride prevents stroke-induced mitochondrial dysfunction. Bevemipretide trihydrochloride is applicable to research related to amyotrophic lateral sclerosis, Parkinson's disease, dementia with Lewy bodies and Huntington's disease.
    Bevemipretide trihydrochloride
  • HY-182648
    K811 1355228-38-4
    K811 is an orally active ASK1 inhibitor with an IC50 of 6 nM. K811 inhibits glial cell activation in the lumbar spinal cord of SOD1G93A transgenic mice. K811 extends the survival of SOD1G93A transgenic mice, a mouse model of amyotrophic lateral sclerosis. K811 can be used in studies related to amyotrophic lateral sclerosis.
    K811