U-Blot® Patched Rabbit mAb
Ucallm_WR5888-50
Price range: $268.00 through $328.00
Product details
Background：
This gene encodes a member of the patched gene family. The encoded protein is the receptor for sonic hedgehog , a secreted molecule implicated in the formation of embryonic structures and in tumorigenesis , as well as the desert hedgehog and indian hedgehog proteins. This gene functions as a tumor suppressor. Mutations of this gene have been associated with basal cell nevus syndrome , esophageal squamous cell carcinoma , trichoepitheliomas , transitional cell carcinomas of the bladder , as well as holoprosencephaly. Alternative splicing results in multiple transcript variants encoding different isoforms. Additional splice variants have been described , but their full length sequences and biological validity cannot be determined currently. [provided by RefSeq , Jul 2008] ,
Specifications
| Size | 50μL/100μL |
| Target | Patched |
| Reactivity | Human |
| Applications | WB, IF, IP, ELISA |
| MW(Calculated) | 161kD |
| MW(Observed) | 180-210kD |
| Host Species | Rabbit |
| Isotype | IgG,Kappa |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | WB 1:500-1:2000; IF 1:200-1:1000; ELISA 1:5000-1:20000; IP 1:50-1:200; |
| Formulation | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| Purification | Recombinant Antibody expressed in animal component-free (ACF) media, purified via Protein A affinity chromatography. |
| Storage | -15°C to -25°C/1 year(Do not lower than -25°C) |
| Concentration | |
| Clonality | Monoclonal |
| Clone Number | RM1602R |
| Immunogen | The specific immunogen used to produce this antibody is proprietary information. |
| Specificity | Endogenous |
| Gene Name | PTCH1 |
| Protein Name | Protein patched homolog 1 |
| Other Name | PTCH1; / PTCH; / Protein patched homolog 1; / PTC; / PTC1 |
| Species | Human |
| Gene ID | 5727 |
| Uniprot | Q13635, |
| Species.1 | Mouse |
| Gene ID.1 | 19206 |
| Uniprot.1 | Q61115 |
| Species.2 | |
| Gene ID.2 | |
| Uniprot.2 | |
| Cellular Localization | Cell membrane; Multi-pass membrane protein . |
| Tissue Expression | In the adult, expressed in brain, lung, liver, heart, placenta, skeletal muscle, pancreas and kidney. Expressed in tumor cells but not in normal skin. |
| Signaling_pathway | Human Diseases >> Cancer: overview >> Pathways in cancer;Human Diseases >> Cancer: specific types >> Basal cell carcinoma;Environmental Information Processing >> Signal transduction >> Hedgehog signaling pathway;Environmental Information Processing >> Signal transduction >> cAMP signaling pathway |
| Research Areas | >>cAMP signaling pathway; / >>Hedgehog signaling pathway; / >>Axon guidance; / >>Pathways in cancer; / >>Proteoglycans in cancer; / >>Basal cell carcinoma |
| Function | developmental stage:In the embryo, found in all major target tissues of sonic hedgehog, such as the ventral neural tube, somites, and tissues surrounding the zone of polarizing activity of the limb bud.,Disease:Defects in PTCH1 are a cause of sporadic basal cell carcinoma (BCC) [MIM:605462].,Disease:Defects in PTCH1 are probably the cause of basal cell nevus syndrome (BCNS) [MIM:109400]; also known as Gorlin syndrome or Gorlin-Goltz syndrome. BCNS is an autosomal dominant disease characterized by nevoid basal cell carcinomas (NBCCS) and developmental abnormalities such as rib and craniofacial alterations, polydactyly, syndactyly, and spina bifida. In addition, the patients suffer from a multitude of tumors like basal cell carcinomas (BCC), fibromas of the ovaries and heart, cysts of the skin, jaws and mesentery, as well as medulloblastomas and meningiomas. PTCH1 is also mutated in squamous cell carcinoma (SCC). Could also be associated with large body size observed in BCNS patients.,Disease:Defects in PTCH1 are the cause of holoprosencephaly type 7 (HPE7) [MIM:610828]. Holoprosencephaly (HPE) [MIM:236100] is the most common structural anomaly of the brain, in which the developing forebrain fails to correctly separate into right and left hemispheres. Holoprosencephaly is genetically heterogeneous and associated with several distinct facies and phenotypic variability.,Function:Acts as a receptor for sonic hedgehog (SHH), indian hedgehog (IHH) and desert hedgehog (DHH). Associates with the smoothened protein (SMO) to transduce the hedgehog's proteins signal. Seems to have a tumor suppressor function, as inactivation of this protein is probably a necessary, if not sufficient step for tumorigenesis.,PTM:Glycosylation is necessary for SHH binding.,similarity:Belongs to the patched family.,similarity:Contains 1 SSD (sterol-sensing) domain.,subunit:Interacts with SNX17.,tissue specificity:In the adult, expressed in brain, lung, liver, heart, placenta, skeletal muscle, pancreas and kidney. Expressed in tumor cells but not in normal skin., |
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