C4 Ceramide is a bioactive sphingolipid and cell-permeable analog of naturally occurring ceramides. [1] [2] [3] It inhibits IL-4 production by 16% in EL4 T cells stimulated with phorbol 12-myristate 13-acetate when used at a concentration of 10 μM. [1] C4 Ceramide is cytotoxic to SK-BR-3 and MCF-7 Adr breast cancer cells (IC50s = 15.9 and 19.9 μM, respectively). [2] C4 Ceramide also increases maturation and stability of cysticfibrosistransmembraneconductanceregulator (CFTR) proteins bearing the F508 deletion (F508del) mutation, enhances cAMP-activated chloride secretion, and suppresses secretion of IL-8 in primary epithelial cells isolated from patients with cysticfibrosis.[3]
Flufenamic acid-d4 is intended for use as an internal standard for the quantification of flufenamic acid by GC- or LC-MS. Flufenamic acid is a non-steroidal anti-inflammatory drug (NSAID) and COX inhibitor (IC50s = 3 and 9.3 µM for human COX-1 and COX-2, respectively). Flufenamic acid inhibits TNF-α-induced increases in COX-2 levels and NF-κB activation in HT-29 colon cancer cells in a concentration-dependent manner. It inhibits calcium influx induced by fMLP or A23187 in human polymorphonuclear leukocytes (PMN) with IC50 values of 29 and 14 µM, respectively. Flufenamic acid also activates various ion channels, including transient receptor potential canonical 6 (TRPC6) and the large-conductance calcium-activated potassium channel (KCa1.1). It also inhibits various ion channels, including TRPC3 and the cysticfibrosistransmembraneconductanceregulator (CFTR). Flufenamic acid (20 mg/kg) reduces increases in intestinal fluid secretion and intestinal barrier disruption in mice ......
CFTR corrector 6 is a highly effective CFTR potentiator utilized in research on cysticfibrosis (CF) and related CFTR-associated disorders[1]. This potent compound exhibits potentiation activity on the CysticFibrosisTransmembraneconductanceRegulator (CFTR), rendering it beneficial for investigations in CF and other associated conditions.
SRI-41315, a chemical compound, effectively addresses premature termination codons (PTCs) associated with cysticfibrosis in immortalized and primary human bronchial epithelial cells. It induces a prolonged pause at stop codons, resulting in the restoration of cysticfibrosistransmembraneconductanceregulator (CFTR) expression and function. This compound achieves PTC suppression by reducing the abundance of the termination factor eRF1. Additionally, SRI-41315 synergistically enhances CFTR activity by potentiating aminoglycoside-mediated readthrough [1].
GLPG2451 is a cysticfibrosistransmembraneconductanceregulator (CFTR) potentiator. It effectively potentiates low temperature rescued F508del CFTR (EC50: 11.1 nM).
GLPG-3221 can be uesd for the treatment of cysticfibrosis.GLPG-3221 is a potent, orally active corrector of CFTR (cysticfibrosistransmembraneconductanceregulator), with an EC50 of 105 nM.