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SUFU Rabbit pAb (bs-11209R)  
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產品編號 bs-11209R
英文名稱 SUFU Rabbit pAb
中文名稱 SUFU抗體
別    名 SU FU; SU(F)U; Su(fu); SUFU_HUMAN; SUFUH; SUFUXL; Suppressor of fused homolog(Drosophila); Suppressor of fused homolog; OTTHUMP00000020374; OTTHUMP00000020377; OTTHUMP00000020379; PRO1280.  
Specific References  (1)     |     bs-11209R has been referenced in 1 publications.
[IF=0] Liu et al. Suppressor of fused (Sufu) represses Gli1 transcription and nuclear accumulation, inhibits glioma cell proliferation, invasion and vasculogenic mimicry, improving glioma chemo-sensitivity and prognosis. (2014) Oncotarget. 5:11681-94  IF,IHC ;  Human.  
研究領域 腫瘤  轉錄調節因子  表觀遺傳學  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應 Mouse,Rat (predicted: Human,Pig,Chicken,Dog,Horse)
產品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 53/106 kDa
檢測分子量
細胞定位 細胞核 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SUFU: 433-484/484 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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.

Function:
Negative regulator in the hedgehog signaling pathway. Down-regulates GLI1-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).

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.

Subcellular Location:
Cytoplasm. Nucleus.

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 fetal brain, lung and kidney.

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.

Similarity:
Belongs to the SUFU family.

SWISS:
Q9UMX1

Gene ID:
51684

Database links:

Entrez Gene: 51684 Human

Omim: 607035 Human

SwissProt: Q9UMX1 Human

Unigene: 404089 Human



產品圖片
Tissue/cell: mouse embryo tissue; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; Incubation: Anti-SUFU Polyclonal Antibody, Unconjugated(bs-11209R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
Tissue/cell: rat brain tissue; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; Incubation: Anti-SUFU Polyclonal Antibody, Unconjugated(bs-11209R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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