Catalog No.: PC-28329Not For Human Use, Lab Use Only.
Claziprotamide (BBP-671) is a potent, brain penetrant small molecule activator of Pantothenate kinase (PanK) with Kd of 97 pM and IC50 of 1.59 nM for PanK3, binds to and activates all PANK isoforms.
Claziprotamide (BBP-671) is a potent, brain penetrant small molecule activator of Pantothenate kinase (PanK) with Kd of 97 pM and IC50 of 1.59 nM for PanK3, binds to and activates all PANK isoforms.
BBP-671 counteracts feedback inhibition of the enzyme to increase the intracellular concentration of CoA.
BBP-671 normalizes the cellular concentrations of liver CoASH and acetyl-CoA in propionic acidemia (PA) mice.
BBP-671 also reduces hpatic propionyl-CoA leading to an improved intracellular C3:C2-CoA ratio.
BBP-671 significantly reduced plasma C3:C2-carnitine ratio and methylcitrate, the hallmark biomarkers of PA.
