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TGFBI Recombinant Rabbit mAb (bsm-54085R)  
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產(chǎn)品編號(hào) bsm-54085R
英文名稱 TGFBI Recombinant Rabbit mAb
中文名稱 角膜上皮蛋白TGFBI重組兔單抗
別    名 AI181842; AI747162; beta ig; beta ig h3; beta ig-h3; BGH3_HUMAN; Big h3; BIGH3; CDB1; CDG2; CDGG1; CSD; CSD1; CSD2; CSD3; EBMD; Kerato epithelin; Kerato-epithelin; LCD1.  
研究領(lǐng)域 細(xì)胞生物  發(fā)育生物學(xué)  神經(jīng)生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  干細(xì)胞  生長(zhǎng)因子和激素  
抗體來源 Rabbit
克隆類型 Recombinant
交叉反應(yīng) Human,Mouse,Rat
產(chǎn)品應(yīng)用 WB=1:500-2000,IHC-P=1:50-200,IHC-F=1:50-200,IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 72 kDa
檢測(cè)分子量
細(xì)胞定位 分泌型蛋白 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human TGFBI 
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(xiàng) 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 an RGD-containing protein that binds to type I, II and IV collagens. The RGD motif is found in many extracellular matrix proteins modulating cell adhesion and serves as a ligand recognition sequence for several integrins. This protein plays a role in cell-collagen interactions and may be involved in endochondrial bone formation in cartilage. The protein is induced by transforming growth factor-beta and acts to inhibit cell adhesion. Mutations in this gene are associated with multiple types of corneal dystrophy. [provided by RefSeq, Jul 2008]

Function:
Binds to type I, II, and IV collagens. This adhesion protein may play an important role in cell-collagen interactions. In cartilage, may be involved in endochondral bone formation.

Subcellular Location:
Secreted > extracellular space > extracellular matrix. May be associated both with microfibrils and with the cell surface. Tissue Specificity : Highly expressed in the corneal epithelium.

Post-translational modifications:
Gamma-carboxyglutamate residues are formed by vitamin K dependent carboxylation. These residues are essential for the binding of calcium.

DISEASE:
Defects in TGFBI are the cause of epithelial basement membrane corneal dystrophy (EBMD) [MIM:121820]; also known as Cogan corneal dystrophy or map-dot-fingerprint type corneal dystrophy. EBMD is a bilateral anterior corneal dystrophy characterized by grayish epithelial fingerprint lines, geographic map-like lines, and dots (or microcysts) on slit-lamp examination. Pathologic studies show abnormal, redundant basement membrane and intraepithelial lacunae filled with cellular debris. Although this disorder usually is not considered to be inherited, families with autosomal dominant inheritance have been identified.
Defects in TGFBI are the cause of corneal dystrophy Groenouw type 1 (CDGG1) [MIM:121900]; also known as corneal dystrophy granular type. Inheritance is autosomal dominant. Corneal dystrophies show progressive opacification of the cornea leading to severe visual handicap.
Defects in TGFBI are the cause of corneal dystrophy lattice type 1 (CDL1) [MIM:122200]. Inheritance is autosomal dominant.
Defects in TGFBI are a cause of corneal dystrophy Thiel-Behnke type (CDTB) [MIM:602082]; also known as corneal dystrophy of Bowman layer type 2 (CDB2). Defects in TGFBI are the cause of Reis-Buecklers corneal dystrophy (CDRB) [MIM:608470]; also known as corneal dystrophy of Bowman layer type 1 (CDB1). Defects in TGFBI are the cause of lattice corneal dystrophy type 3A (CDL3A) [MIM:608471]. CDL3A clinically resembles to lattice corneal dystrophy type 3, but differs in that its age of onset is 70 to 90 years. It has an autosomal dominant inheritance pattern.
Defects in TGFBI are the cause of Avellino corneal dystrophy (ACD) [MIM:607541]. ACD could be considered a variant of granular dystrophy with a significant amyloidogenic tendency. Inheritance is autosomal dominant.

Similarity:
Contains 1 EMI domain.
Contains 4 FAS1 domains.

SWISS:
Q15582

Gene ID:
7045

產(chǎn)品圖片
Western blot analysis of TGFBI on different lysates with Rabbit anti-TGFBI antibody (bsm-54085R) at 1/500 dilution. Lane 1: Mouse eyeball tissue lysate Lane 2: Mouse colon tissue lysate Lysates/proteins at 20 μg/Lane. Predicted band size: 75 kDa Observed band size: 68 kDa Exposure time: 2 minutes; 10% SDS-PAGE gel.
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