Human GLi3 ELISA Kit (RMEK-0152639)
Cat. No.: RMEK-0152639
Category: ELISA Kits
INQUIRY
1 x 96 tests
Human GLi3 ELISA Kit is an in vitro enzyme-linked immunosorbent assay for the quantitative measurement of human GLi3 in serum, plasma, and cell culture supernatants. This assay employs an antibody specific for human GLi3 coated on a 96-well plate.
Product Features
| Species Reactivity | Human |
|---|---|
| Detection Method | Colorimetric |
| Assay Duration | Multiple steps standard assay |
| Assay Type | Sandwich (quantitative) |
| Sensitivity | Sensitivity: 40 pg/mL |
| Range | 40 pg/mL - 10000 pg/mL |
| Sample Type | Cell culture supernatant, Plasma, Serum |
| Recovery | Serum-81.93-68% - 100%; Plasma-71.92-67% - 82%; Cell culture media-90.23-85% - 97% |
| Key Features | Sensitivity: 40 pg/mL; Range: 40 pg/mL - 10000 pg/mL; Sample type: Cell culture supernatant, Plasma, Serum; Detection method: Colorimetric; Assay type: Sandwich (quantitative); Reacts with: Human |
Target Information
| Target Symbol | GLI3 |
|---|---|
| UniProt ID | P10071 |
| Biomarker of SCs/CSCs | Non Small Cell Lung Cancer |
| Function | Has a dual function as a transcriptional activator and a repressor of the sonic hedgehog (Shh) pathway, and plays a role in limb development. The full-length GLI3 form (GLI3FL) after phosphorylation and nuclear translocation, acts as an activator (GLI3A) while GLI3R, its C-terminally truncated form, acts as a repressor. A proper balance between the GLI3 activator and the repressor GLI3R, rather than the repressor gradient itself or the activator/repressor ratio gradient, specifies limb digit number and identity. In concert with TRPS1, plays a role in regulating the size of the zone of distal chondrocytes, in restricting the zone of PTHLH expression in distal cells and in activating chondrocyte proliferation. Binds to the minimal GLI-consensus sequence 5'-GGGTGGTC-3'. |
| Cellular Localization | Nucleus. Cytoplasm. Cell projection > cilium. GLI3FL is localized predominantly in the cytoplasm while GLI3R resides mainly in the nucleus. Ciliary accumulation requires the presence of KIF7 and SMO. Translocation to the nucleus is promoted by interaction with ZIC1. |
| Post-transcriptional Modifications | Phosphorylated on multiple sites by protein kinase A (PKA) and phosphorylation by PKA primes further phosphorylation by CK1 and GSK3. Phosphorylation is essential for its proteolytic processing.Transcriptional repressor GLI3R, a C-terminally truncated form, is generated from the full-length GLI3 protein (GLI3FL/GLI3-190) through proteolytic processing. This process requires PKA-primed phosphorylation of GLI3, ubiquitination of GLI3 and the presence of BTRC. GLI3FL is complexed with SUFU in the cytoplasm and is maintained in a neutral state. Without the Hh signal, the SUFU-GLI3 complex is recruited to cilia, leading to the efficient processing of GLI3FL into GLI3R. GLI3R formation leads to its dissociation from SUFU, allowing it to translocate into the nucleus, and repress Hh target genes. When Hh signaling is initiated, SUFU dissociates from GLI3FL and this has two consequences. First, GLI3R production is halted. Second, free GLI3FL translocates to the nucleus, where it is phosphorylated, destabilized, and converted to a transcriptional activator (GLI3A). Phosphorylated in vitro by ULK3. |
| Involvement in Disease | Defects in GLI3 are the cause of Greig cephalo-poly-syndactyly syndrome (GCPS). GCPS is an autosomal dominant disorder affecting limb and craniofacial development. It is characterized by pre- and postaxial polydactyly, syndactyly of fingers and toes, macrocephaly and hypertelorism. Defects in GLI3 are a cause of Pallister-Hall syndrome (PHS). PHS is characterized by a wide range of clinical manifestations. It mainly associates central or postaxial polydactyly, syndactyly, and hypothalamic hamartoma. Malformations are frequent in the viscera, e. g. anal atresia, bifid uvula, congenital heart malformations, pulmonary or renal dysplasia. It is an autosomal dominant disorder. Defects in GLI3 are a cause of type A1/B postaxial polydactyly (PAPA1/PAPB). PAPA in humans is an autosomal dominant trait characterized by an extra digit in the ulnar and/or fibular side of the upper and/or lower extremities. The extra digit is well formed and articulates with the fifth, or extra, metacarpal/metatarsal, and thus it is usually functional. Defects in GLI3 are a cause of polydactyly preaxial type 4 (POP4). Polydactyly preaxial type 4 (i. e. , polydactyly on the radial/tibial side of the hand/foot) covers a heterogeneous group of entities. In preaxial polydactyly type IV, the thumb shows only the mildest degree of duplication, and syndactyly of various degrees affects fingers 3 and 4. Defects in GLI3 are the cause of acrocallosal syndrome (ACS); also abbreviated ACLS. ACS is characterized by postaxial polydactyly, hallux duplication, macrocephaly, and absence of the corpus callosum, usually with severe developmental delay. |
Storage & Shipping
| Storage | Store at 2-8°C |
|---|---|
| Shipping | Gel Packs |
| Stability | The stability of kit is determined by the loss rate of activity. The loss rate of this kit is less than 5% within the expiration date under appropriate storage condition. |
For research use only. Not for clinical use.
