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Rabbit Anti-Integrin alpha 6/FITC Conjugated antibody
background:
Integrins are important extracellular matrix (ECM) receptor proteins located on cell surfaces. They are hetrodimers composed of an alpha and a beta transmembrane glycoprotein subunit. Around twenty two different integrins (different alpha/ beta subunit combinations) are found in nature. Integrins are generally present in high concentrations at the cell surface, but, unlike most other cell surface receptors, they bind ligands with very low affinity. Due to their weak individual binding, integrins need to cluster and bind in groups in order to effectively bind the ECM. Integrins bind many different ligands including laminin. Each integrin is made up of a large N terminal extracellular domain that binds the ECM ligand and a small C terminal cytoplasmic domain that mediates interaction with the actin cytoskeleton and signaling function. Integrin alpha 6 complexes are receptors for laminins, which are components of basement membranes. Integrin alpha 6 complexes may play an important role in embryogenesis.
Function:
Integrin alpha-6/beta-1 is a receptor for laminin on platelets. Integrin alpha-6/beta-4 is a receptor for laminin in epithelial cells and it plays a critical structural role in the hemidesmosome.
Subunit:
Heterodimer of an alpha and a beta subunit. The alpha subunit is composed of an heavy and a light chain linked by a disulfide bond. Alpha-6 associates with either beta-1 or beta-4. Interacts with HPS5. Interacts with RAB21.
Subcellular Location:
Cell membrane; Single-pass type I membrane protein.
Tissue Specificity:
Integrin alpha-6/beta-4 is predominantly expressed by epithelia. Isoforms containing segment X1 are ubiquitously expressed. Isoforms containing segment X1X2 are expressed in heart, kidney, placenta, colon, duodenum, myoblasts and myotubes, and in a limited number of cell lines; they are always coexpressed with the ubiquitous isoform containing segment X1. In some tissues (e.g. Salivary gland), isoforms containing cytoplasmic segment A and isoforms containing segment B are detected while in others, only isoforms containing one cytoplasmic segment are found (segment A in epidermis and segment B in kidney). [PTM] Isoforms containing segment A, but not segment B, are the major targets for PMA-induced phosphorylation. Phosphorylation occurs on 'Ser-1103' of isoform alpha-6X1X2A. Phosphorylation is not required for the induction of integrin alpha-6A/beta-1 high affinity but may reduce the affinity for ligand.
Post-translational modifications:
Isoforms containing segment A, but not segment B, are the major targets for PMA-induced phosphorylation. Phosphorylation occurs on 'Ser-1103' of isoform alpha-6X1X2A. Phosphorylation is not required for the induction of integrin alpha-6A/beta-1 high affinity but may reduce the affinity for ligand.
DISEASE:
Defects in ITGA6 are a cause of epidermolysis bullosa letalis with pyloric atresia (EB-PA) [MIM:226730]; also known as junctional epidermolysis bullosa with pyloric atresia (PA-JEB) or aplasia cutis congenita with gastrointestinal atresia. EB-PA is an autosomal recessive, frequently lethal, epidermolysis bullosa with variable involvement of skin, nails, mucosa, and with variable effects on the digestive system. It is characterized by mucocutaneous fragility, aplasia cutis congenita, and gastrointestinal atresia, which most commonly affects the pylorus. Pyloric atresia is a primary manifestation rather than a scarring process secondary to epidermolysis bullosa.
Similarity:
Belongs to the integrin alpha chain family.
Contains 7 FG-GAP repeats.
Database links:
Entrez Gene: 3655 Human
Entrez Gene: 11283 Mouse
Omim: 147556 Human
SwissProt: P23229 Human
SwissProt: Q61739 Mouse
Unigene: 133397 Human
Unigene: 225096 Mouse
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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