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.
![Immunocytochemistry Integrin beta 4/CD104 Antibody (422325) [Unconjugated]](http://images.novusbio.com/fullsize2/Integrin_beta_4_MAB4060_Immunocytochemistry__Immunofluorescence_19916.jpg)
