
Journal of Medicinal Chemistry p. 3634 - 3664 (2020)
Update date:2022-08-15
Topics:
Di Martino, Simona
Tardia, Piero
Cilibrasi, Vincenzo
Caputo, Samantha
Mazzonna, Marco
Russo, Debora
Penna, Ilaria
Realini, Natalia
Margaroli, Natasha
Migliore, Marco
Pizzirani, Daniela
Ottonello, Giuliana
Bertozzi, Sine Mandrup
Armirotti, Andrea
Nguyen, Duc
Sun, Ying
Bongarzone, Ernesto R.
Lansbury, Peter
Liu, Min
Skerlj, Renato
Scarpelli, Rita
Sphingolipids (SphLs) are a diverse class of molecules that are regulated by a complex network of enzymatic pathways. A disturbance in these pathways leads to lipid accumulation and initiation of several SphL-related disorders. Acid ceramidase is one of the key enzymes that regulate the metabolism of ceramides and glycosphingolipids, which are important members of the SphL family. Herein, we describe the lead optimization studies of benzoxazolone carboxamides resulting in piperidine 22m, where we demonstrated target engagement in two animal models of neuropathic lysosomal storage diseases (LSDs), Gaucher's and Krabbe's diseases. After daily intraperitoneal administration at 90 mg kg-1, 22m significantly reduced the brain levels of the toxic lipids glucosylsphingosine (GluSph) in 4L;C? mice and galactosylsphingosine (GalSph) in Twitcher mice. We believe that 22m is a lead molecule that can be further developed for the correction of severe neurological LSDs where GluSph or GalSph play a significant role in disease pathogenesis.
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