|
ORF cDNA clones
|
CRISPR / TALEN
|
Lentivirus
|
AAV
|
TALE-TF
|
ORF knockin clones
|
|
Antibody
|
Proteins
|
miRNA target clones
|
qPCR primers
|
shRNA clones
|
miRNA products
|
Promoter clones
|
Validated All-in-One™ qPCR Primer for SHH(NM_000193.3) Search again
Product ID:
HQP088425
(click here to view gene annotation page)
Powered by BiozSpecies:
Human
Symbol:
Alias:
HHG1, HLP3, HPE3, MCOPCB5, SMMCI, ShhNC, TPT, TPTPS
Gene Description:
sonic hedgehog signaling molecule
Target Gene Accession:
NM_000193.3(click here to view gene page)
Estimated Delivery:
Approximately 1-3 weeks, but may vary. Please email sales@genecopoeia.com or call 301-762-0888 to confirm ETA.
Important Note:
By default, qPCR primer pairs are designed to measure the expression level of the splice variant (accession number) you selected for this gene WITHOUT consideration of other possible variants of this gene. If this gene has multiple variants, and you would like to measure the expression levels of one particular variant, multiple variants, or all variants, please contact us for a custom service project at inquiry@genecopoeia.com.
Validated result:
|
|
| A | B |
|
Each All-in-One™ qPCR Primer is experimentally validated to yield a single dissociation curve peak and to generate a single amplification of the correct size for the targeted gene. A cDNA Pool, containing reverse transcript products of 8 different human tissue total RNA(Liver, Testis,Muscle,Thyroid,Brain,Spleen,Stomach,Small Intestine)),was used as the qPCR validation template. qPCR was performed using 0.2 µM primer with 2XAll-in-One™ qPCR Mix (Catalog#: QP001,QP002,QP004,QP005). Reactions were incubated for 10min. at 95°C, followed by 40 cycles of 95°C for 10 sec.; 60°C, 20 sec. and 72°C, 15sec. using Bio-Rad iQ5™ Instrument. At the end of the last cycle, temperature was increased from 72 to 95°C to produce a melting curve. Panel A: Validated Result for Amplification Curve; Panel B: Validated Result for Melting Curve. |
|
Summary
This gene encodes 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].
Gene References into function
- While the gene contributes to face development, it is not a frequent cause of cleft lip or palate.
- role in activating poliovirus receptor/CD155 gene
- characterization of (SHH) expression in the human fetal prostate
- Shh-mediated signaling is a physiological component of T cell responses, which acts to modulate CD4+ T cell effector function.
- REVIEW: Hedgehog signaling in human disease
- induces capillary morphogenesis by endothelial cells through phosphoinositide 3-kinase
- The Tbx1 regulatory region was responsive to signaling by Sonic hedgehog (Shh) in vivo.
- Down-reuglation of this protein's expression in pulmonary hypoplasia is associated with congenital diaphragmatic hernia.
- Role for hedgehog signaling in regeneration and carcinogenesis of airway epithelium
- Aberrant Shh signaling may play a critical role in the pathogenesis of esophageal atresia with tracheoesophageal fistula in humans.
- These studies are the first demonstration that mutations in SHH associated with human HPE (holoprosencephaly) perturb the in vivo patterning function of SHH in the developing nervous system.
- deregulated hedgehog signaling in the genesis of basaloid follicular hamartomas
- Adriamycin influences the Shh signalling pathway, resulting in disruption of normal development of the foregut.
- mutations in a long-range Shh enhancer located in an intron of the limb region 1 gene result in preaxial polydactyly
- model of the interactions between beta-catenin and hedgehog signaling in the epidermis in which SHH promotes proliferation of progenitors of the hair lineages whereas IHH stimulates proliferation of sebocyte precursors
- In gray matter from brains of multiple sclerosis (MS) victims, the total amount of Shh was less than normals and the signaling 20 kDa protein was greatly reduced.
- a wide range of digestive tract tumours, including most of those originating in the oesophagus, stomach, biliary tract and pancreas, but not in the colon, display increased Hh pathway activity, which is suppressible by cyclopamine, a Hh pathway antagonis
- Hedgehog pathway may have an early and critical role in the genesis of pancreatic cancer; maintenance of hedgehog signalling is important for aberrant proliferation and tumorigenesis
- Decreased expression of the Shh pathway in atrophic gastritis and gastric cancer might reflect altered differentiation processes within the gastric unit and contributes to the development of gastric atrophy.
- Shh signaling has a role in the cellular proliferation of oral squamous cell carcinoma cells
- signaling molecules might play a role in epithelial-mesenchymal interactions and cell proliferation in tooth development as well as in growth of [ameloblastomas]
- SHH signalling can increase aberrant cell survival in colorectal tumour cells.
- Thirteen novel SHH mutations found in a cohort of Holoprosencephaly patinets.
- Overexpression of a wild-type regulatory subunit of protein kinase A is sufficient to activate sonic hedgehog target gene transcription.
- Results identify REN(KCTD11) as a suppressor of Hedgehog signaling and suggest that its inactivation might lead to a deregulation of the tumor-promoting Hedgehog pathway in medulloblastoma.
- sonic hedgehog mutations have a role in holoprosencephaly
- Data report expression of sonic hedgehog-GLI-1 pathway components in adult human prostate cancer, often with enhanced levels in tumors versus normal prostatic epithelia.
- Shh down-regulated in the precursor cell population the expression of IL-7R as well as stromal-derived factor-1 chemokine receptor, CXCR4, and inhibited IL-7-dependent STAT5 phosphorylation.
- SHH signaling in inflammatory diseases of the gut acts to ensure stem cell restitution of damaged mucosal epithelium.
- Taken together, our data suggest that the hedgehog pathway is weakly active in normal adult urothelial cells and of limited importance in transitional cell carcinoma.
- ptc1 mutation enhances a downstream reporter of sonic hedgehog (shh)-ptc1 signaling.
- an intact EGF signaling axis cooperates with shh and is a critical mediator of matrix invasion in a tumor type characterized by disrupted shh.
- Elevated expression of hedgehog target genes human patched gene 1 (PTCH1) or Gli1 occurs in 63 of the 99 primary gastric cancers.
- regulatory elements around the SHH gene may contribute to the maintenance of conserved synteny across human chromosome 7q36.3
- elevated expression of Shh and its target genes is quite common in esophageal cancers
- Shh might maintain pituitary cells in nonproliferative state. Shh is newly described hypophysiotropic cytokine and its down-regulation may be involved in pathogenesis of pituitary adenomas.
- SHH gene therapy may have considerable therapeutic potential in individuals with acute and chronic myocardial ischemia.
- The effects of seven missense mutations associated with holoprosencephaly that affect the N-terminal signaling domain of SHH have been characterized
- This review highlights the hypothesis of hedgehog pathway activation in damaged nerve tissue, inducing the repair process, as a potentially new approach to treatment of neurodegenerative diseases and dysfunction, including spinal cord injury.
- Hedgehog signaling is conserved in hepatic progenitors from fetal development through adulthood and may be a new therapeutic target in patients with liver damage.
- A pattern of strong Shh expression with weak expression of Foxf1 in all cases of UIP (usual interstitial pneumonitis) and a complementary expression of Shh and Foxf1 in cases of nonspecific interstitial pneumonitis (NSIP-F).
- Rsults suggested that the increased and constitutive Shh expression is implicated in gastric carcinogenesis, and that promoter methylation may be an important regulatory mechanism of Shh expression.
- Transgenic SHH signals cooperate with those of FGF9 to regulate mesenchymal proliferation in distinct submesothelial and subepithelial regions of developing lung.
- Shh is an important regulator of human putative epidermal stem cell proliferation in the basal layer of fetal epidermis and modulates the cell responsiveness to EGF
- Increased expression of SHh mRNA in human colonic adenocarcinomas and in a colorectal cell line.
- SHH morphogens are associated with microvesicles (MVs) shed from the plasma membrane of apoptotic/stimulated T cells.
- beta-catenin might be involved in the Hh signaling pathway via enhancement of the transcriptional activity of GLI
- analysis of Hedgehog modulator properties after functional coupling of Smoothened to G15
- Expressions of Shh and Gli-1 were significantly higher in leiomyomas than in gastrointestinal stromal tumors. Expressions of Ptc and Smo did not correlate with histopathological differentiation.
- sFRP-1, a target gene of the hedgehog pathway, is involved in cross-talk between the hedgehog pathway and the Wnt pathway
- The results presented in this study implicate p63 in the regulation of the Shh signaling pathway.
- Dignaling is clearly upregulated in gastrointestinal neuroendocrine carcinomas in a Gli1-dependent manner.
- Mutations within ZRS of sonic hedgehog appear to be a common cause of familial triphalangeal thumb and preaxial polydactyly.
- Results suggest that the SHH pathway may be a target to overcome MDR and increase chemotherapeutic response.
- The metabolic pathway of SHH might have been responsible for the left side-restricted multiple fetal abnormalities reported.
- The stronger Shh expression in intestinal metaplasia and gastric adenoma indicates that hedgehog protein is involved at an early phase of gastric carcinogenesis.
- Melanomas require SHH-GLI signaling regulated by interactions bewteen GLI1 and the RAS-MEK/AKT pathways.
- data support a direct molecular link between the Hh signaling pathway and SOX9 regulation, wherein SHH stimulates SOX9 through its mediator GLI1, and are consistent with a mechanism of SOX9 regulation through distal chromatin interactions
- The quantitative expression of sHh signaling detected by Western blotting in pancretic cancer.
- Shh-Ptch1-Gli1 signaling pathway may play a role in the progression of colorectal tumor.
- Shh signaling enhanced K-Ras-mediated pancreatic tumorigenesis and reduced the dependence of tumor cells on the sustained activation of Ras-stimulated signaling pathways.
- Expression of Shh ligand by glioblastoma multiforme cells is a mechanism for Hh pathway activation. Shh mRNA was found in many primary tumor samples, the highest level of expression being in the tumor with the most elevated Gli1 level.
- Activin B is a potent inducer of Pdx1 as well as Shh in differentiating embryonic stem cell derived embryoid bodies
- Protein kinase A, not Epac, suppresses hedgehog activity and regulates glucocorticoid sensitivity in acute lymphoblastic leukemia cells
- Snail and SHH are overexpressed in a large subset of neuroendocrine tumors of the ileum.
- we focus on the SHh signaling pathway and its role in gastrointestinal (GI) tract development and maintenance and consider the diseases resulting from aberrant SHh activity--REVIEW
- The congenital abnormality preaxial polydactylya is caused by ectopic expression of the Sonic Hedgehog (SHH) in the developing limb bud.
- are the first to suggest a role and mechanism for Shh signaling as it relates to the metastatic potential of gastric cancer, thereby indicating potential therapeutic molecular targets to decrease metastasis
- A novel microduplication in 7q36.3 results in a similar triphalangeal thumb and polysyndactyly phenotype as caused by single nucleotide alterations in the ZRS, the limb specific SHH regulatory element.
- In the adult stomach, Shh is expressed and secreted from the acid-producing parietal cells, where it is believed to play an essential role in gastric tissue homeostasis and normal differentiation of the epithelium.
- These findings show that human embryonic stem cells contain primary cilia associated with working Hedgehog machinery.
- Point mutations in the ZPA regulatory sequence (ZRS), a long range cis-regulator for the SHH gene, and duplications encompassing the ZRS cause distinctive limb phenotypes.
- Overexpression of sonic hedgehog is associated with angiogenesis in pancreatic cancer
- SHH and Gli1 mRNAs are likely to be up-regulated from adenoma and from borderline to carcinoma cells, respectively, in intraductal papillary mucinous neoplasm of the pancreas
- A dominant mutation in the sonic hedgehog regulatory sequence is associated with the common cause of triphalangeal thumb in southern England.
- These results indicate a significant role for Shh-GLI signaling in the proliferation of mantle cell lymphoma.
- Both the Shh precursor and mature protein are N-palmitoylated by Hhat, and the reaction occurs during passage through the secretory pathway
- the onset of Hh signaling from human embryogenesis to fetal development is associated with accumulation of Hh signaling components Smo and Gli2 in pancreatic duct primary cilia and a reduction of Gli3 in the duct epithelium
- Tested variants are not associated with anorectal malformations and most are predicted to be benign.
- Conjunctival epithelium constitutively expresses a low level of Shh, and its expression increases during malignant conversion of epithelial cells.
- Oncogenic Hh signaling promotes medulloblastoma from lineage-restricted granule cell progenitors.
- Pint mutations in patients with type 2 diabetes and their families were studied. mitochondrial genes including np3316, np3394 and np3426 in the ND1 region and np3243 in the tRNA(Leu(UUR))were screened.
- The role of NF-kappaB in SHH expression, and the implications for cancer therapy, are reported.
- investigate the involvement of the SSH pathway in Smith-Lemli-Opitz syndrome-affected individuals developing tumors
- Down-regulation of SHH signaling pathway activity is involved in 5-fluorouracil-induced apoptosis and mobility inhibition in Hep3B cells.
- its signal transduction regulates tumor development. (review)
- SHH contributes to the formation of desmoplasia in pancreatic cancer
- Magnetic resonance imaging monitors physiological changes with antihedgehog therapy in pancreatic adenocarcinoma xenograft model.
- NRP-1 knockdown RCC cells exhibit a more differentiated phenotype, as evidenced by the expression of epithelial-specific and kidney-specific cadherins, and the inhibition of sonic hedgehog expression participated in this effect
- Shh signaling regulates Sufu activity by inducing its turnover via the ubiquitin-proteasome system.
- Hh targets CATB, and Hh signaling through CATB might influence pancreatic cancer cell invasiveness
- human tumor suppressor SUFU has a role in Hedgehog signaling [review]
- Results show that SHH holoprosencephaly missense mutations affect SHH biogenesis and signaling at multiple steps, which results in low levels of protein expression, defective processing of SHH into its active form and protein with reduced activity.
- Hh signaling is important in the pathogenesis of B-CLL and, hence, may be a potential therapeutic target
- Hedhog traget genes show increased transcript levels in hepatoblastoma samples.