产品信息(Information):
Known as: | Sonic Hedgehog Signaling Molecule, Autoprocessing Domains, Shh Unprocessed N-Terminal Signaling And C-Terminal, MCOPCB5, HHG1, TPTPS, SMMCI, HLP3, TPT |
Source: | Homo sapiens (Human) |
Cat.No.: | Y03902 |
Construction: | Cys198-Ser462 |
Tag: | His tag (C-terminus) |
Molecular Mass: | 28.51 kDa |
Expression Host: | HEK293 cells |
背景介绍(Background):
This protein is a protein that is instrumental in patterning the early embryo. It has been implicated as the key inductive signal in patterning of the ventral neural tube, the anterior-posterior limb axis, and the ventral somites. Of three human proteins showing sequence and functional similarity to the sonic hedgehog protein of Drosophila, this protein is the most similar. The protein is made as a precursor that is autocatalytically cleaved; the N-terminal portion is soluble and contains the signalling activity while the C-terminal portion is involved in precursor processing. More importantly, the C-terminal product covalently attaches a cholesterol moiety to the N-terminal product, restricting the N-terminal product to the cell surface and preventing it from freely diffusing throughout the developing embryo. Defects in this protein or in its signalling pathway are a cause of holoprosencephaly (HPE), a disorder in which the developing forebrain fails to correctly separate into right and left hemispheres. HPE is manifested by facial deformities. It is also thought that mutations in this gene or in its signalling pathway may be responsible for VACTERL syndrome, which is characterized by vertebral defects, anal atresia, tracheoesophageal fistula with esophageal atresia, radial and renal dysplasia, cardiac anomalies, and limb abnormalities. Additionally, mutations in a long range enhancer located approximately 1 megabase upstream of this gene disrupt limb patterning and can result in preaxial polydactyly. [provided by RefSeq, Jul 2008]
Sonic hedgehog (SHH) is involved in the development of various organs during embryogenesis. It is expressed in the central nervous system, lungs, teeth, intestines, and hair follicles during development. SHH, initially synthesized as a 45 kDa precursor, is thus cleaved into a 19 kDa (SHH-N) and a 26 kDa (SHH-C) secreted peptide. SHH-N is modified by the addition of lipid (palmitoyl and cholesterol) and mediates signaling in vertebrates and invertebrates. SHH-C is believed to possess any biological activity under physiological conditions. Hedgehog ligands bind to Patched receptors and derepress the GPCR SMO. Hedgehog signals are then transduced to the canonical SMO-SUFU-GLI and non-canonical SMO-Gi-RhoA signaling cascades. Canonical Hedgehog/GLI signaling up-regulates the transcription of target genes, such as BCL2, FOXA2, FOXE1, FOXF1, FOXL1, FOXM1, GLI1, HHIP, MYCN, NANOG, PTCH1, PTCH2, SFRP1, SNAI1 (Snail), SOX2 and WNT2B, in a cellular context-dependent manner. Non-canonical SMO-Gi-RhoA signaling promotes cytoskeletal reorganization and cellular motility and potentiates GLI1-dependent transcription. The Hedgehog signaling-independent activation of MTOR-S6K1 and MEK-ERK signaling enhances GLI-dependent transcription. Canonical and non-canonical Hedgehog signaling cascades, as well as Hedgehog-independent GLI signaling cascades, converge on the GLI transcription network. The activation of Hedgehog signaling in tumor cells leads to proliferation, self-renewal, survival, and invasion through the GLI-dependent transcription network, hypoxia-dependent epithelial-to-mesenchymal transition (EMT), and RhoA-dependent cytoskeletal reorganization.

图 SHH信号通路图
制剂(Formulation):
Lyophilized from 0.22 μm filtered solution in PBS,5%mannital,0.01% Tween80 pH7.4.
质量控制(Quality Control):
Purity: ≥ 95% as determined by reducing SDS-PAGE.
Endotoxin: < 0.1 EU/ug as determined by LAL test.
保存(Storage):
Use a manual defrost freezer and avoid repeated freeze - thaw cycles.
12 months from date of receipt, -20 to -70℃ as supplied.
1 month, 2 to 8℃ under sterile conditions after reconstitution.
6 months, -20 to -70℃ under sterile conditions after reconstitution.
FOR RESEARCH USE ONLY
参考文献:
[1] Katoh M. et al. 2019. Clin Sci (Lond). 133(8):953-970.
[2] Lézot F, et al. 2020. Cells. 9(3):536.
[3] Skoda AM, et al. 2018. Bosn J Basic Med Sci. 18(1):8-20.