Hedgehog (Hh) is composed of N-terminal and C-terminal domains that dissociate in a self-catalyzed proteolytic cleavage reaction. The N-terminal product HhNp, modified by cholesterol during self-cleavage, harbors all known Hh signaling activities. When synthesized in the absence of the C-terminal domain (and hence lacking cholesterol modification), the N-terminal domain is aberrantly targeted and released selectively into the retina.Hedgehog signaling pathway is linked to tumorigenesis and is aberrantly activated in a variety of cancers. Hh ligands bind to and suppress the transmembrane receptor Patched (PTCH), which suppresses Smoothened (SMO), a seven-transmembrane-helix protein that positively regulates the Hh pathway.Sonic hedgehog (Shh) is a morphogen essential to the developing nervous system that continues to play an important role in adult life by contributing to cell proliferation and differentiation, maintaining blood-brain barrier integrity, and being cytoprotective against oxidative and excitotoxic stress, all features of importance in amyotrophic lateral sclerosis (ALS).
||(R)-DRF053 dihydrochloride (DRF053) is a potent, cell-permeable, dual CK1/CDK inhibitor with IC50 of 14 nM, 220 nM and 80 nM for CK1, CDK5/p25 and CDK1/cyclin B, respectively.
||Vismodegib is an orally active hedgehog pathway inhibitor with an IC50 of 3 nM and also inhibits P-gp, ABCG2 with IC50 values of 3.0 μM and 1.4 μM, respectively.
||SANT-1 directly binds to Smoothened (Smo) receptor with Kd of 1.2 nM and inhibits Smo agonist effects with IC50 of 20 nM.
||Cyclopamine is a Hedgehog (Hh) pathway antagonist with IC50 of 46 nM in the Hh cell assay.
||RU-SKI 43 HCl
||RU-SKI 43 Hcl is a small molecule inhibitor of Hhat(Hedgehog acyltransferase), the enzyme responsible for the attachment of palmitate onto Shh.
||RU-SKI 43 is a small molecule inhibitor of Hhat(Hedgehog acyltransferase), the enzyme responsible for the attachment of palmitate onto Shh.
||Ciliobrevin A is a hedgehog (Hh) signaling pathway inhibitor with median inhibitory concentration (IC50) less than 10 μM.
||JK184 is a potent Hedgehog (Hh) pathway inhibitor with IC50 of 30 nM in mammalian cells.
||Jervine(11-Ketocyclopamine) is a naturally occuring steroidal alkaloid that causes cyclopia by blocking sonic hedgehog(Shh) signaling; Jervine is an inhibitor of Smo.
||HPI-1 is a Hedgehog (Hh) pathway inhibitor that suppresses signaling through Shh (IC50=1.5 uM) without significantly affecting Wnt signaling (IC50>30 uM).
||AY-9944 is a cholesterol biosynthesis inhibitor that targets the 7-dehydro cholesterol Δ7-reductase (DHCR7) enzyme (IC50=13 nM), attenuates of Smoothened-dependent and -independent Hh signaling.
||A potent, selective and cell active Hedgehog acyltransferase (Hhat) inhibitor with IC50 of 0.87 uM.
||A highly potent, orally active hedgehog signaling (Hh) inhibitor with IC50 of 4.4 nM in Gli-luc reporter assays.
||Cyclopamine tartrate is a natural steroidal alkaloid that inhibits hedgehog (Hh) signaling with IC50 of 46 nM.
||Robotnikinin is asmall molecule that binds the extracellular Sonic Hedgehog (Shh) protein (Kd=3.1 uM) and blocks Shh-signaling in cell lines, human primary keratinocytes and synthetic model of human skin.