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Rabbit Anti-EFEMP1/BF594 Conjugated antibody (bs-14516R-BF594)
訂購熱線:400-901-9800
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-14516R-BF594
英文名稱 Rabbit Anti-EFEMP1/BF594 Conjugated antibody
中文名稱 BF594標(biāo)記的原纖維蛋白樣蛋白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.  
規(guī)格價(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 腫瘤  神經(jīng)生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  細(xì)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 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 53kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human EFEMP1
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產(chǎn)品介紹 background:
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.

Database links:

Entrez Gene: 2202 Human

Entrez Gene: 216616 Mouse

Entrez Gene: 305604 Rat

Omim: 601548 Human

SwissProt: Q12805 Human

SwissProt: Q8BPB5 Mouse

SwissProt: O35568 Rat

Unigene: 76224 Human

Unigene: 44176 Mouse

Unigene: 163265 Rat



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