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FBN3 Rabbit pAb (bs-16034R)  
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產品編號 bs-16034R
英文名稱 FBN3 Rabbit pAb
中文名稱 FBN3蛋白抗體
別    名 Fibrillin 3; FBN3_HUMAN; KIAA1776.  
研究領域 細胞生物  信號轉導  結合蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human)
產品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ICC/IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 297 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 FBN3: 2701-2809/2809 
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 This gene encodes a protein that belongs to the fibrillin gene family. Fibrillins are extracellular matrix molecules that assemble into microfibrils in many connective tissues. This gene is most highly expressed in fetal tissues and its protein product is localized to extracellular microfibrils of developing skeletal elements, skin, lung, kidney, and skeletal muscle. This gene is potentially involved in Weill-Marchesani syndrome. While several transcript variants may exist for this gene, their full-length natures have not been described to date. [provided by RefSeq, Jul 2008]

Function:
Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. Fibrillin-containing microfibrils provide long-term force bearing structural support.

Subcellular Location:
Secreted; extracellular space; extracellular matrix.

Tissue Specificity:
Predominantly expressed in connective tissues such as skeletal muscle, tendon, skin, perichondrium and periosteum. Highly expressed in fetal lung, brain, kidney. Expressed at low level in prostate, testis, mammary gland, uterus, ovary, placenta, bladder, adrenal gland, thyroid, fetal thymus, fetal liver, liver, fetal heart and heart.

Post-translational modifications:
Probably forms intermolecular disulfide bonds either with other FBN3 molecules or with other components of the microfibrils (By similarity).

Similarity:
Belongs to the fibrillin family.
Contains 44 EGF-like domains.
Contains 9 TB (TGF-beta binding) domains.

SWISS:
Q75N90

Gene ID:
84467

Database links:

Entrez Gene: 84467 Human

Omim: 608529 Human

SwissProt: Q75N90 Human

Unigene: 370362 Human



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