| 產(chǎn)品編號 | bs-14516R |
| 英文名稱 | EFEMP1 Rabbit pAb |
| 中文名稱 | 原纖維蛋白樣蛋白DHRD抗體 |
| 別 名 | DHRD; Doyne honeycomb retinal degeneration; DRAD; EFEMP 1; EFEMP1; EGF containing fibulin like extracellular matrix protein 1; EGF containing fibulin like extracellular matrix protein 1 precursor; EGF-containing fibulin-like extracellular matrix protein 1; Epidermal growth factor containing fibulin like extracellular matrix protein 1; Extracellular protein S1 5; Extracellular protein S1-5; FBLN 3; FBLN3; FBLN3_HUMAN; FBNL; FIBL 3; FIBL-3; FIBL3; Fibrillin like; Fibrillin like protein; Fibrillin-like protein; FIBULIN 3; Fibulin-3; Fibulin3; FLJ35535; Heat shock 70 KD protein 1; MGC111353; MLVT; MTLV; S1 5; T16 protein. |
| 研究領(lǐng)域 | 腫瘤 神經(jīng)生物學(xué) 信號轉(zhuǎn)導(dǎo) 細胞外基質(zhì) |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Mouse (predicted: Human,Rat) |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 53 kDa |
| 檢測分子量 | |
| 細胞定位 | 細胞漿 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human EFEMP1: 401-493/493 |
| 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(chǎn)品介紹 |
This gene encodes a member of the fibulin family of extracellular matrix glycoproteins. Like all members of this family, the encoded protein contains tandemly repeated epidermal growth factor-like repeats followed by a C-terminus fibulin-type domain. This gene is upregulated in malignant gliomas and may play a role in the aggressive nature of these tumors. Mutations in this gene are associated with Doyne honeycomb retinal dystrophy. Alternatively spliced transcript variants that encode the same protein have been described.[provided by RefSeq, Nov 2009] Function: Binds EGFR, the EGF receptor, inducing EGFR autophosphorylation and the activation of downstream signaling pathways. May play a role in cell adhesion and migration. May function as a negative regulator of chondrocyte differentiation. In the olfactory epithelium, it may regulate glial cell migration, differentiation and the ability of glial cells to support neuronal neurite outgrowth. Subcellular Location: Secreted > extracellular space. Secreted > extracellular space > extracellular matrix. Localizes to the lamina propria underneath the olfactory epithelium. Tissue Specificity: In the eye, associated with photoreceptor outer and inner segment regions, the nerve fiber layer, outer nuclear layer and inner and outer plexiform layers of the retina. DISEASE: Defects in EFEMP1 are a cause of Doyne honeycomb retinal dystrophy (DHRD) [MIM:126600]; also known as malattia leventinese (MLVT) (ML). DHRD is an autosomal dominant disease characterized by yellow-white deposits known as drusen that accumulate beneath the retinal pigment epithelium. Similarity: Belongs to the fibulin family. Contains 6 EGF-like domains. SWISS: Q12805 Gene ID: 2202 Database links: Entrez Gene: 2202 Human Entrez Gene: 216616 Mouse Omim: 601548 Human SwissProt: Q12805 Human SwissProt: Q8BPB5 Mouse Unigene: 76224 Human Unigene: 44176 Mouse Unigene: 163265 Rat |
| 產(chǎn)品圖片 |
Protein: lung(mouse) lysate at 40ug;
Primary: rabbit Anti-EFEMP1 (bs-14516R) at 1:300;
Secondary: HRP conjugated Goat-Anti-rabbit IgG(bs-0295G-HRP) at 1: 5000;
Predicted band size: 53 kD
Observed band size: 49 kD
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