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FRAS1 Rabbit pAb (bs-13212R)  
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50ul/1180.00元
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產品編號 bs-13212R
英文名稱 FRAS1 Rabbit pAb
中文名稱 細胞外基質蛋白FRAS1抗體
別    名 Extracellular matrix protein FRAS1; Fras 1; Fras1; FRAS1_HUMAN; Fraser syndrome 1.  
研究領域 心血管  細胞生物  信號轉導  細胞外基質  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應 (predicted: Human,Mouse,Rat)
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 440 kDa
檢測分子量
細胞定位 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human FRAS1: 1101-1200/4008 <Extracellular>
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 an extracellular matrix protein that appears to function in the regulation of epidermal-basement membrane adhesion and organogenesis during development. Mutations in this gene cause Fraser syndrome, a multisystem malformation that can include craniofacial, urogenital and respiratory system abnormalities. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Oct 2009].

Subcellular Location:
Cell membrane; Single-pass type I membrane protein; Extracellular side (Potential).

Tissue Specificity:
Expressed in many adult tissues, with highest levels in kidney, pancreas and thalamus. Relatively high expression was also detected in fetal kidney and heart.

DISEASE:
Defects in FRAS1 are a cause of Fraser syndrome (FRASS) [MIM:219000]. Fraser syndrome is a multisystem malformation usually comprising cryptophthalmos, cutaneous syndactyly, ear abnormalities, renal agenesis and congenital heart defects.

Similarity:
Belongs to the FRAS1 family.
Contains 5 Calx-beta domains.
Contains 12 CSPG (NG2) repeats.
Contains 14 FU (furin-like) repeats.
Contains 6 VWFC domains.

SWISS:
Q86XX4

Gene ID:
80144

Database links:

Entrez Gene: 80144 Human

Omim: 607830 Human

SwissProt: Q86XX4 Human

Unigene: 369448 Human



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