SUFU/Suppressor of Fused,抑制Hh信号通路蛋白SUFU抗体

产品编号HZ-11209R
英文名称SUFU/Suppressor of Fused
中文名称抑制Hh信号通路蛋白SUFU抗体
别 名SU FU; SU(F)U; Su(fu); SUFU; SUFU_HUMAN; SUFUH; SUFUXL; Suppressor of fused homolog (Drosophila); Suppressor of fused homolog; OTTHUMP00000020374; OTTHUMP00000020377; OTTHUMP00000020379; PRO1280.
说 明 书0.1ml 0.2ml
研究领域肿瘤 转录调节因子 表观遗传学
抗体来源Rabbit
克隆类型Polyclonal
交叉反应 Human, Mouse, Rat, Chicken, Dog, Pig, Horse,
SUFU/Suppressor of Fused,抑制Hh信号通路蛋白SUFU抗体产品应用ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量53, 106kDa
细胞定位细胞核 细胞浆
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human SUFU(433-485 aa)
亚 型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. 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.
SUFU/Suppressor of Fused,抑制Hh信号通路蛋白SUFU抗体PubMedPubMed
产品介绍background:
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.

SUFU/Suppressor of Fused,抑制Hh信号通路蛋白SUFU抗体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.

Database links:
Entrez Gene: 51684 Human
Omim: 607035 Human
SwissProt: Q9UMX1 Human
Unigene: 404089 Human

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

产品图片

Protein: line1,mouse intestinal lysates, 60ug; line 2,mouse lung 60ug;line3,mouse brain Primary: Anti-SUFU (HZ-11209R) at 1:300;  Secondary: HRP conjugated Goat-Anti-Rabbit IgG(HZ-0295G) at 1: 5000;  ECL excitated the fluorescence;  Predicted band size :52 kD Observed band size :52 kD 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(HZ-11209R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining 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