Recombinant Human Suppressor of fused homolog (SUFU)

Code CSB-YP891560HU
MSDS
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Source Yeast
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Code CSB-EP891560HU
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Source E.coli
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Code CSB-EP891560HU-B
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Source E.coli
Conjugate Avi-tag Biotinylated
E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.
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Code CSB-BP891560HU
MSDS
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Source Baculovirus
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Code CSB-MP891560HU
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Source Mammalian cell
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Product Details

Purity
>85% (SDS-PAGE)
Target Names
SUFU
Uniprot No.
Alternative Names
OTTHUMP00000020374; OTTHUMP00000020377; OTTHUMP00000020379; PRO1280; SU FU; SU(F)U; Su(fu); SUFU; SUFU negative regulator of hedgehog signaling; SUFU_HUMAN; SUFUH; SUFUXL; Suppressor of Fused; Suppressor of fused homolog (Drosophila); Suppressor of fused homolog
Species
Homo sapiens (Human)
Expression Region
1-484
Target Protein Sequence
MAELRPSGAP GPTAPPAPGP TAPPAFASLF PPGLHAIYGE CRRLYPDQPN PLQVTAIVKY WLGGPDPLDY VSMYRNVGSP SANIPEHWHY ISFGLSDLYG DNRVHEFTGT DGPSGFGFEL TFRLKRETGE SAPPTWPAEL MQGLARYVFQ SENTFCSGDH VSWHSPLDNS ESRIQHMLLT EDPQMQPVQT PFGVVTFLQI VGVCTEELHS AQQWNGQGIL ELLRTVPIAG GPWLITDMRR GETIFEIDPH LQERVDKGIE TDGSNLSGVS AKCAWDDLSR PPEDDEDSRS ICIGTQPRRL SGKDTEQIRE TLRRGLEINS KPVLPPINPQ RQNGLAHDRA PSRKDSLESD SSTAIIPHEL IRTRQLESVH LKFNQESGAL IPLCLRGRLL HGRHFTYKSI TGDMAITFVS TGVEGAFATE EHPYAAHGPW LQILLTEEFV EKMLEDLEDL TSPEEFKLPK EYSWPEKKLK VSILPDVVFD SPLH
Protein Length
full length protein
Tag Info
Tag type will be determined during the manufacturing process.
The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
Form
Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
Buffer before Lyophilization
Tris/PBS-based buffer, 6% Trehalose, pH 8.0
Reconstitution
We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
Troubleshooting and FAQs
Storage Condition
Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
Shelf Life
The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
Lead Time
Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
Notes
Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet
Please contact us to get it.

Customer Reviews and Q&A

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Target Background

Function
Negative regulator in the hedgehog/smoothened signaling pathway. Down-regulates GLI1-mediated transactivation of target genes. Down-regulates GLI2-mediated transactivation of target genes. Part of a corepressor complex that acts on DNA-bound GLI1. May also act by linking GLI1 to BTRC and thereby targeting GLI1 to degradation by the proteasome. Sequesters GLI1, GLI2 and GLI3 in the cytoplasm, this effect is overcome by binding of STK36 to both SUFU and a GLI protein. Negative regulator of beta-catenin signaling. Regulates the formation of either the repressor form (GLI3R) or the activator form (GLI3A) of the full-length form of GLI3 (GLI3FL). 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. When Hh signaling is initiated, SUFU dissociates from GLI3FL and the latter translocates to the nucleus, where it is phosphorylated, destabilized, and converted to a transcriptional activator (GLI3A). Required for normal embryonic development. Required for the proper formation of hair follicles and the control of epidermal differentiation.
Gene References into Functions
  1. Down-regulation of microRNA-224 inhibits growth and epithelial-to-mesenchymal transition phenotype via modulating SUFU expression in bladder cancer cells. PMID: 28780419
  2. Itch/beta-arrestin2 complex binds SuFu and induces its Lys63-linked polyubiquitylation without affecting its stability. PMID: 29515120
  3. Patients with SUFU pathogenic variants were significantly more likely to develop a medulloblastoma, a meningioma or an ovarian fibroma, but were less likely to develop a jaw cyst. PMID: 28596197
  4. Nek2A is found to stabilize SuFu at least partly depending on its kinase activity, thereby triggering phosphorylation of the SuFu protein. PMID: 27297360
  5. Extra-mitochondrial prosurvival BCL-2 proteins regulate gene transcription by inhibiting the SUFU tumour suppressor. PMID: 28945232
  6. Missense mutation in SUFU gene is associated with Joubert Syndrome with Cranio-facial and Skeletal Defects. PMID: 28965847
  7. In conclusion, this study showed the potential of miR-342-3p as a therapeutic target to promote bone regeneration by modulating expression of Sufu in UCMSCs. PMID: 28765042
  8. miRNA-194 is oncogenic and promotes gastric cancer cell proliferation and migration by activating Wnt signaling, at least in part, via suppression of SUFU. PMID: 27810403
  9. SUFU's role in Hedgehog signaling, tumor progression, and highlight a way in which BCCs can arise PMID: 28030567
  10. In summary, these findings reveal Fbxl17 as a novel regulator of the Hedgehog signaling pathway and highlight the perturbation of the Fbxl17-Sufu axis in the pathogenesis of medulloblastoma. PMID: 27234298
  11. there was a positive correlation between VDR status and the expression of Suppressor of fused gene (SuFu), a hedgehog pathway inhibitor. miR-214 on the other hand suppressed SuFu protein expression. PMID: 27693451
  12. The data indicate that there exists a novel transcript variant of SUFU which can be transcribed and translated into corresponding protein and its transcription is related with metastasis of lymph nodes in pancreatic ductal adenocarcinoma. PMID: 27840902
  13. We showed that the supplementation of the osteogenic differentiation medium with PTHrP inhibited the alkaline phosphatase activity and the expression of the transcription factor DLX3, but the depletion of PTHrP did not support the differentiation of DFCs.We showed that SUFU (Suppressor Of Fused Homolog) was not regulated during the osteogenic differentiation in DFCs PMID: 27368119
  14. This study uncovers a previously unappreciated miR-214-Sufu pathway in controlling EMT and metastasis of lung adenocarcinoma. PMID: 26462018
  15. A germline SUFU mutation was present in a patient with MHIBCC, and additional acquired SUFU mutations underlie the development of infundibulocystic basal cell carcinomas PMID: 26677003
  16. SUFU germline polymorphism is associated with acute GVHD PMID: 26067905
  17. Sufu has a role in repressing Gli1 transcription and nuclear accumulation, inhibiting glioma cell proliferation, invasion and vasculogenic mimicry, improving glioma chemo-sensitivity and prognosis PMID: 25373737
  18. suggest childhood brain magnetic resonance imaging surveillance is justified in SUFU-related, but not PTCH1-related, Gorlin syndrome PMID: 25403219
  19. SUFU polymorphisms are associated with neural tube defects in a high-risk population in China. PMID: 24070372
  20. Functionally, RIOK3 acts as a SUFU-dependent positive regulator of Hedgehog signaling. PMID: 24018050
  21. Data indicate a significant role of hedgehog receptor PTCH1 and SUFU in the pathogenesis of keratocystic odontogenic tumor (KCOT). PMID: 23951062
  22. It is demonstrated that GLI binding is associated with major conformational changes in SUFU, including an intrinsically disordered loop that is also crucial for pathway activation. PMID: 24311597
  23. The 'closed' form of Sufu is stabilized by Gli binding and inhibited by Hh treatment, whereas the 'open' state of Sufu is promoted by Gli-dissociation and Hh signaling. PMID: 24217340
  24. Polymorphisms in the SUFU gene (encoding for a negative regulator of the hedgehog signaling pathway) are associated with protection from Enterobacteriacea bacteremia related organ injury and sepsis severity. PMID: 23538333
  25. SUFU germline mutation is associated with nevoid basal cell carcinoma syndrome associated with meningioma. PMID: 22829011
  26. Our genetic and functional analyses indicate that germline mutations in SUFU also predispose to meningiomas, particularly to multiple meningiomas. PMID: 22958902
  27. mechanism of action on Hedgehog signaling by a suppressor of fused carboxy terminal variant PMID: 22666390
  28. germline SUFU mutations are responsible for a high proportion of desmoplastic medulloblastoma in children younger than 3 years of age PMID: 22508808
  29. Rab23 directly associates with Su(Fu) and inhibits Gli1 function in a Su(Fu)-dependent manner. PMID: 22365972
  30. inactivating germline mutations of SUFU cause ~2-3% of sporadic medulloblastomas and > 10% of desmoplastic medulloblastomas. PMID: 21188540
  31. We found that hedgehog-interacting protein, PDGFRalpha, smoothened and Su(Fu) gene highly expressed in the primary esophageal squamous cell carcinomas PMID: 21210262
  32. Glis3 interacts with Suppressor of Fused (SUFU) PMID: 21543335
  33. ciliary localization of Sufu is dependent on ciliary-localized Gli proteins, and is inhibited by PKA activation PMID: 21209912
  34. Dual Phosphorylation of suppressor of fused (Sufu) by PKA and GSK3beta regulates its stability and localization in the primary cilium. PMID: 21317289
  35. Suppressor of fused transgene negatively regulates hedgehog signaling and proliferation in retinal progenitor cells (RPCs); unexpectedly, Sufu-null RPCs fail to maintain their multipotency, differentiating as amacrine and horizontal neurons. PMID: 21451052
  36. Germline SUFU mutations were identified in two families with several children under 3 years of age diagnosed with medulloblastoma. PMID: 19833601
  37. Suppressor of Fused represses Gli-mediated transcription by recruiting the SAP18-mSin3 corepressor complex. PMID: 11960000
  38. Mutations in SUFU predispose to medulloblastoma. PMID: 12068298
  39. loss of SUFU function results in overactivity of both the Sonic Hedgehog, and the WNT signaling pathways, leading to excessive proliferation and failure to differentiate resulting in medulloblastomas. PMID: 15077159
  40. Overexpression of SUFU is associated with Split-hand/split-foot malformation 3 PMID: 16691619
  41. A role for SIL in derepressing GLI1 from the negative control of SUFU. PMID: 18829525
  42. Shh signaling regulates Sufu activity by inducing its turnover via the ubiquitin-proteasome system. PMID: 18997815
  43. expression induced by 4-Hydroxynonenal,one of several lipid oxidation products, in human leukemia cells, HL-60; this tumor suppressor protein is a target of miR-378 PMID: 19022373
  44. human tumor suppressor SUFU has a role in Hedgehog signaling [review] PMID: 19055941
  45. Suppressor of fused was overexpressed in endometrial carcinoma compared with the hyperplastic endometrium PMID: 19432668
  46. First report of a germ line SUFU mutation associated with Gorlin syndrome. PMID: 19533801

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Involvement in disease
Medulloblastoma (MDB); Joubert syndrome 32 (JBTS32)
Subcellular Location
Cytoplasm. Nucleus.
Protein Families
SUFU family
Tissue Specificity
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 feta
Database Links

HGNC: 16466

OMIM: 155255

KEGG: hsa:51684

STRING: 9606.ENSP00000358918

UniGene: Hs.404089

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