Home > Product > Antibody > Rabbit Anti-CCBE1/FITC Conjugated antibody
CCBE 1; ccbe1; CCBE1_HUMAN; Collagen and calcium binding EGF domain containing protein 1; Collagen and calcium binding EGF domains 1; Collagen and calcium-binding EGF domain-containing protein 1; Full of fluid protein homolog.
Cat:
SL7985R-FITC
Species Reactivity:
(predicted: Human,Mouse,Rat,Dog,Pig,Horse,Zebrafish)
Immunogen:
KLH conjugated synthetic peptide derived from human CCBE1
Format:
Lyophilized or Liquid
Storage instructions:
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 ant
Buffer:
0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Concentration:
1mg/ml
Clonality:
Polyclonal
Isotype:
IgG
Applications:
IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.
Host:
Rabbit
Calculated MW:
41kDa
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Unit:
Price: $
Product PDFs
Datasheet:


background:
This gene is thought to function in extracellular matrixremodeling and migration. It is predominantly expressed in theovary, but down regulated in ovarian cancer cell lines and primarycarcinomas, suggesting its role as a tumour suppressor. Mutationsin this gene have been associated with Hennekamlymphangiectasia-lymphedema syndrome, a generalized lymphaticdysplasia in humans. [provided by RefSeq, Mar 2010].

Function:
Required for lymphangioblast budding and angiogenicsprouting from venous endothelium during embryogenesis.

Subcellular Location:
Secreted (Potential).

DISEASE:
Defects in CCBE1 are the cause of Hennekamlymphangiectasia-lymphedema syndrome (HLLS) [MIM:235510]. HLLS is ageneralized lymph-vessels dysplasia characterized by intestinallymphangiectasia with severe lymphedema of the limbs, genitalia andface. In addition, affected individuals have unusual facies andsevere mental retardation.

Similarity:
Belongs to the CCBE1 family. Contains 1 EGF-like domain.

Database links:
UniProtKB/Swiss-Prot: Q6UXH8.1

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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