sclerosis

SRI-011381 hydrochloride, a new-type agonist of the TGF-beta signaling pathway, is utilized in the treatment of Alzheimer's disease.

Tauroursodeoxycholate (UR 906), also known as ursodoxicoltaurine, is a highly hydrophilic tertiary bile acid that is produced in humans at a low concentration. Tauroursodeoxycholate is the more hydrophilic form of ursodeoxycholic acid, which is the more abundant naturally produced bile acid in humans.Tauroursodeoxycholate is being investigated for use in several conditions such as Primary Biliary Cirrhosis (PBC), insulin resistance, amyloidosis, Cystic Fibrosis, Cholestasis, and Amyotrophic Lateral Sclerosis.

DNL343 is a selective and potent eIF2B activator that crosses the blood-brain barrier and inhibits the assembly of stress granules (SGs) induced by the C9ORF72 dipeptide repeats. DNL343 may be useful in the study of neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS).

Aminaftone, a derivative of 4-aminobenzoic acid, inhibits endothelin-1 (ET-1) production and is used to study hypertension and systemic sclerosis.

Utreloxastat (PTC857) is a novel 15-lipoxygenase inhibitor that can be used to study amyotrophic lateral sclerosis .

Silperisone HCl (RGH-5002) blocks sodium and calcium channels in cells, decreases excitability and contractility of muscle cells, reduces peripheral tone, and acts as a muscle relaxant and peripheral vasodilator. Silperisone HCl is used to treat recurrent painful myoclonus due to spinal cord injury, abnormal hypertonia due to cerebrovascular disease, dystonia symptoms, cone tension syndrome, multiple sclerosis myospasm and myelitis. silperisone is a sodium channel protein type 2 alpha channel blocker. silperisone is an organosilicon similar to tolperisone, an organosilicon compound with centrally acting muscle relaxant properties.

DZ2002, an orally active, reversible, and low-cytotoxic type III SAHH inhibitor (Ki=17.9 nM), exhibits significant immunosuppressive activity. It effectively prevents experimental dermal fibrosis by reversing the profibrotic phenotype in multiple cell types, making it useful for studying autoimmune diseases like lupus syndrome and systemic sclerosis.

(2R,3S)-N-(4-(2,6-dimethoxyphenyl)-5-(5-methylpyridin-3-yl)-4H-1,2,4-triazol-3-yl)-3-(5-methylpyrimidin-2-yl)butane-2-sulfonamide is an Apelin receptor agonist with an EC50 for Apelin receptors of 0.012 µM.(2R,3S)-N-(4-(2,6-dimethoxyphenyl)-5-(5-methylpyridin-3-yl)-4H-1,2,4-triazol-3-yl)-3-(5- methylpyrimidin-2-yl)butane-2-sulfonamide is used in the treatment of heart failure, atherosclerosis, obesity, diabetes mellitus, amyotrophic lateral sclerosis, coronary artery disease, hypertension, stroke and myocardial infarction.

CYM5181 is related to the body's immune system and can be used to study multiple sclerosis, transplant rejection, and adult respiratory distress syndrome.

Firategrast (SB 683699) is an orally active and specific α4β1 α4β7 integrin antagonist that reduces the transport of lymphocytes into the central nervous system (CNS) and decreases multiple sclerosis (MS) activity. It is used in relapsing-remitting multiple sclerosis, associated with modest reductions in CD4, CD8, and CD19 lymphocyte counts in cerebrospinal fluid.

Antroquinonol is a ubiquinone derivative from Antrodia camphorata with anti-inflammatory and anticancer activities.Antroquinonol reduces oxidative stress and inhibits inflammation and sclerosis in mice with focal segmental glomerulosclerosis through enhancement of the Nrf2 signaling pathway.Antroquinonol has potential for use in the treatment of non-small cell lung cancer. Antroquinonol has potential for the treatment of non-small cell lung cancer and may be used in colon cancer research.

Dimethyl fumarate (DMF) is an Nrf2 activator with oral activity and blood-brain barrier permeability. Dimethyl fumarate has antimicrobial, anti-inflammatory, and immunomodulatory activities and has been used in the study of multiple sclerosis.

Baclofen (Lioresal) is a synthetic chlorophenyl-butanoic acid derivative used to treat spasms due to spinal cord damage and multiple sclerosis, muscle-relaxing Baclofen acts as a gamma-aminobutyric acid (GABA) agonist specific for GABA-B receptors. It acts at spinal and supraspinal sites, reducing excitatory transmission.

Tolperisone hydrochloride (Muscalm), a centrally acting muscle relaxant, is used in the therapy of pathologically increased tone of the cross-striated muscle resulted by neurological diseases (damage of the encephalomyelitis, myelopathy, multiple sclerosis, pyramidal tract) and of spastic paralysis and other encephalopathies manifested with muscular dystonia.

Teriflunomide (A 77-1726) is the principal active metabolite of leflunomide, an approved therapy for rheumatoid arthritis and multiple sclerosis.

Cladribine (2CdA), an adenosine deaminase inhibitor, is utilized in the treatment of lymphoproliferative diseases.

Riluzole (RP-54274) is a glutamate antagonist (RECEPTORS, GLUTAMATE) used as an anticonvulsant (ANTICONVULSANTS) and to prolong the survival of patients with AMYOTROPHIC LATERAL SCLEROSIS.

BN201 (OCS-05) is a drug with neuroprotective and myelin repair effects, which acts mainly by activating serum glucocorticoid kinase-2 (SGK2), which in turn activates the FOXO3 pathway to promote neuronal survival and myelin repair. It is mainly used for the treatment of inflammatory neurological diseases such as multiple sclerosis (MS) and experimental autoimmune encephalomyelitis (EAE).BN201 is able to regulate multiple kinases in the insulin growth factor 1 (IGF1) pathway and cross the blood-brain barrier.

CP-456773 (MCC950 (CP-456773) and CRID3) is an effective and specific cytokine release inhibitor and NLRP3 inflammasome inhibitor. CP-456773 inhibits IL-1β secretion and caspase 1 processing. MCC950 blocked canonical and noncanonical NLRP3 activation at nanomolar concentrations. MCC950 specifically inhibited activation of NLRP3 but not the AIM2, NLRC4 or NLRP1 inflammasomes. MCC950 reduced IL-1β production in vivo and attenuated the severity of experimental autoimmune encephalomyelitis (EAE), a disease model of multiple sclerosis.

BAF312 (Siponimod (BAF-312)), a next-generation S1P receptor modulator, is specific for S1P1 and S1P5 receptors with EC50 of 0.39 nM and 0.98 nM, respectively. The specificity of BAF312 for S1P1 and S1P5 receptors exhibits >1000-fold over S1P2, S1P3 and S1P4 receptors.

Icerguastat (IFB-088) is a selective inhibitor of holophosphatase. Sephin1 safely and selectively inhibited a regulatory subunit of protein phosphatase 1 in vivo.

SOD1-Derlin-1 inhibitor-1 (SOD1-Derlin-1 Inhibitor 56-20) (compound 56-20) is an inhibitor of SOD1-Derlin-1 interaction. SOD1-Derlin-1 inhibitor-1 inhibits SOD1 G93A -Derlin-1 complex with an IC 50 value of 7.11 μM. SOD1-Derlin-1 inhibitor-1 can be used for amyotrophic lateral sclerosis research[1].

MTP 131 is a mitochondria-targeted peptide antioxidant.1,2It localizes to the mitochondria and reducestert-butyl hydroperoxide-induced lipid peroxidation and apoptosis in SH-SY5Y cells when used at concentrations ranging from 0.001 to 1 nM.1MTP 131 (2 mg kg) reduces infarct volume, hemispheric swelling, and glutathione (GSH) depletion in a mouse model of acute cerebral ischemia induced by middle cerebral artery occlusion (MCAO).2It increases survival, improves motor function, and decreases degeneration of the lumbar spinal cord in a superoxide dismutase 1 mutant (SOD1G93A) transgenic mouse model of amyotrophic lateral sclerosis (ALS) when administered at a dose of 5 mg kg. MTP 131 reduces albuminuria, urinary hydrogen peroxide levels, and mesangial matrix accumulation, as well as preserves superoxide production, in adb dbmouse model of diabetic nephropathy.3 1.Zhao, K., Luo, G., Giannelli, S., et al.Mitochondria-targeted peptide prevents mitochondrial depolarization and apoptosis induced by tert-butyl hydroperoxide in neuronal cell linesBiochem. Pharmacol.70(12)1796-1806(2005) 2.Szeto, H.H.Mitochondria-targeted peptide antioxidants: Novel neuroprotective agentsAAPS J.8(3)E521-E531(2006) 3.Miyamoto, S., Zhang, G., Hall, D., et al.Restoring mitochondrial superoxide levels with elamipretide (MTP-131) protects db db mice against progression of diabetic kidney diseaseJ. Biol. Chem.295(21)7249-7260(2020)

PDGFR Tyrosine Kinase Inhibitor III (PDGF Receptor Tyrosine Kinase Inhibitor III) is a multikinase inhibitor that inhibits PDGFR, EGFR, FGFR, PKA, and PKC and can be used for research on amyotrophic lateral sclerosis [1].