Integrin beta 4 is a glycoprotein which associates with the a6 integrin to form the a6/b4 complex. Integrin alpha 6/beta 4 is a receptor for laminin. It plays a critical structural role in the hemidesmosome of epithelial cells. Defects in Integrin beta 4 gene are a cause of epidermolysis bullosa letalis with pyloric atresia (EB PA); also known as junctional epidermolysis bullosa with pyloric atresia (PA-JEB) or aplasia cutis congenita with gastrointestinal atresia. EB-PA is characterized by mucocutaneous fragility and gastrointestinal atresia, which most commonly affects the pylorus. Moreover, defects in Integrin beta 4 gene are a cause of generalized atrophic benign epidermolysis bullosa (GABEB). This nonlethal form of junctional epidermolysis bullosa is characterized by life long blistering of the skin, associated with hair and tooth abnormalities.
Specificity
Reacts with Human beta4 integrin. The antigen is present on epithelial cells.
Application
Stains human skin in immunofluorescence at approximately 1:200; acetone fixation required.
Also suitable for use in immunoprecipitation and flow cytometric assays.
Optimal working dilutions must be determined by the end user.
This Anti-Integrin β4 Antibody, clone 3E1 is validated for use in FC, IC, IH, IP for the detection of Integrin β4.
Physical form
UnPurified ascites containing no preservatives.
Other Notes
Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.
Legal Information
CHEMICON is a registered trademark of Merck KGaA, Darmstadt, Germany
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Storage Class
10 - Combustible liquids
wgk_germany
WGK 1
flash_point_f
Not applicable
flash_point_c
Not applicable
Certificates of Analysis (COA)
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