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SUFU Rabbit pAb
- 产品详情
- 实验流程
Application 
| IHC-P, IHC-F, IF |
|---|---|
| Primary Accession | Q9UMX1 |
| Other Accession | Q9UMX1 |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 53947 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human SUFU |
| Epitope Specificity | 433-484/484 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SIMILARITY | Belongs to the SUFU family. |
| SUBUNIT | May form homodimers. Part of a DNA-bound corepressor complex containing SAP18, GLI1 and SIN3. Part of a complex containing CTNNB1. Binds BTRC, GLI2, GLI3, SAP18 and STK36. Binds both free and DNA-bound GLI1. Interacts with KIF7. Interacts with GLI3FL and this interaction regulates the formation of either repressor or activator forms of GLI3. Its association with GLI3FL is regulated by Hh signaling and dissociation of the SUFU-GLI3 interaction requires the presence of the ciliary motor KIF3A. Interacts with ULK3; inactivating the protein kinase activity of ULK3. |
| DISEASE | Defects in SUFU are a cause of medulloblastoma (MDB) [MIM:155255]. MDB is a malignant, invasive embryonal tumor of the cerebellum with a preferential manifestation in children. Defects in SUFU play a role in predisposition to desmoplastic MDB. These tumors make up about 20 to 30% of medulloblastomas, have a more nodular architecture than 'classical' medulloblastoma, and may have a better prognosis. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | SUFU is a key negative regulator in the vertebrate Hedgehog signaling pathway. SUFU interacts with genes encoding proteins in this signal transduction pathway. In Drosophila, Intracellular transduction of the Hedgehog pathway involves the release of a large complex containing SUFU. SUFU inhibits the activity of the transcription factor Gli1 and interacts with Gli2, Gli3 and the serine/threonine kinase Fused. SUFU is widely expressed in adult and embryonic tissues with higher expression in tissues patterned by hegdehog signaling. The SUFU gene locus maps to a region that is deleted in glioblastomas, prostate cancer, malignant melanoma and endometrial cancer. |
| Gene ID | 51684 |
|---|---|
| Other Names | BCNS2; JBTS32; PRO1280; SUFUH; SUFUXL; SUFU_HUMAN; SUFU; SUFU negative regulator of hedgehog signaling; suppressor of fused homolog (Drosophila) |
| Dilution | IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500 |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
For Research Use Only. Not For Use In Diagnostic Procedures.
| Name | SUFU {ECO:0000303|PubMed:12068298, ECO:0000312|HGNC:HGNC:16466} |
|---|---|
| Function | Negative regulator in the hedgehog/smoothened signaling pathway that acts by sequestering the GLI (GI1, GLI2 and GLI3) transcription factors in the cytoplasm (PubMed:10559945, PubMed:10564661, PubMed:10806483, PubMed:12068298, PubMed:12975309, PubMed:15367681, PubMed:22365972, PubMed:24217340, PubMed:24311597, PubMed:27234298, PubMed:28965847, PubMed:35831023). When smoothened signaling is initiated, GLI transcription factors dissociate from SUFU, translocate to the nucleus and activate expression of target genes (PubMed:24311597, PubMed:28965847, PubMed:35831023). In absence of smoothened signaling, the SUFU-GLI3 complex is recruited to cilia, leading to the efficient processing of full-length GLI3 into transcription repressor GLI3R: SUFU participates to GLI3 processing by promoting recruitment of GSK3B to GLI3 (PubMed:24311597, PubMed:28965847). May also act as a negative regulator of beta-catenin signaling (By similarity). Required for normal embryonic development (By similarity). Required for the proper formation of hair follicles and the control of epidermal differentiation (By similarity). |
| Cellular Location | Cytoplasm. Nucleus. Cell projection, cilium {ECO:0000250|UniProtKB:Q9Z0P7}. Note=Recruited to cilia in response to smoothened signaling, leading to dissociation from GLI (GI1, GLI2 and GLI3) transcription factors. {ECO:0000250|UniProtKB:Q9Z0P7} |
| Tissue Location | Ubiquitous in adult tissues. Detected in osteoblasts of the perichondrium in the developing limb of 12-week old embryos. Isoform 1 is detected in fetal brain, lung, kidney and testis Isoform 2 is detected in fetal testis, and at much lower levels in fetal brain, lung and kidney. |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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