Arimoclomol
CAS No. : 289893-25-0
(Synonyms: BRX-220 (free base))
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| Cat. No. : | HY-106443 |
| M.Wt: | 313.78 |
| Formula: | C14H20ClN3O3 |
| Purity: | >98 % |
| Solubility: | DMSO : 25 mg/mL (ultrasonic) |
Arimoclomol (BRX-220 free base) is an orally active co-inducer of heat shock proteins (HSP). Arimoclomol protects motor neurons by enhancing Hsp expression, thus directly affecting protein aggregation and clearance of misfolded assemblies via the proteasome-ubiquitin system.Arimoclomol can be used for the study of Niemann–Pick disease type C. Arimoclomol can cross the blood-brain barrier and can be used in research on Niemann-Pick disease type C[1][2][3].
IC50 & Target:HSP[1]
In Vivo:Arimoclomol (BRX-220; 20 mg/kg; orally administered for 5 d) has a protective effect against cholecystokinin-octapeptide (CCK)-induced acute pancreatitis[1].
In the SOD1-G93A mouse model of amyotrophic lateral sclerosis, arimoclomol can delay muscle denervation[2].
Arimoclomol has been used in preclinical studies for neurodegenerative lysosomal storage diseases, such as Niemann-Pick disease type C (NPC), and has shown improvement in the core pathological lesions of central nervous system (CNS) involvement in Gaucher disease (GD2, GD3)[2].
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