Synthesized peptide derived from human ITGA2B (light chain form 1, Cleaved-Gln891)

This antibody detects endogenous levels of Human ITGA2B (light chain form 1, Cleaved-Gln891, protein was cleaved amino acid sequence between 890-891 ). It doesn't recognize light chain form 2

ITGA2B GP2B ITGAB

ITGA2B (light chain form 1, Cleaved-Gln891)

Integrin alpha-IIb ;
GPalpha IIb ;
GPIIb ;
Platelet membrane glycoprotein IIb ;
CD antigen CD41 ;
[Cleaved into: Integrin alpha-IIb heavy chain ;
Integrin alpha-IIb light chain, form 1 ;
Integrin alpha-IIb light chain, form 2]

This gene encodes a member of the integrin alpha chain family of proteins. The encoded preproprotein is proteolytically processed to generate light and heavy chains that associate through disulfide linkages to form a subunit of the alpha-IIb/beta-3 integrin cell adhesion receptor. This receptor plays a crucial role in the blood coagulation system, by mediating platelet aggregation. Mutations in this gene are associated with platelet-type bleeding disorders, which are characterized by a failure of platelet aggregation, including Glanzmann thrombasthenia. [provided by RefSeq, Jan 2016],

Disease:Defects in ITGA2B are a cause of Glanzmann thrombasthenia (GT) [MIM:273800]; also known as thrombasthenia of Glanzmann and Naegeli. This autosomal recessive disorder is the most common inherited disease of platelets. GT is characterized by mucocutaneous bleeding of mild-to-moderate severity and the inability of this integrin to recognize macromolecular or synthetic peptide ligands. GT has been classified clinically into types I and II. In type I, platelets show absence of the glycoprotein IIb/beta-3 complexes at their surface and lack fibrinogen and clot retraction capability. In type II, the platelets express the glycoprotein IIb/beta-3 complex at reduced levels (5-20% controls), have detectable amounts of fibrinogen, and have low or moderate clot retraction capability. The platelets of GT 'variants' have normal or near normal (60-100%) expression of dysfunctional receptors.,Function:Integrin alpha-IIb/beta-3 is a receptor for fibronectin, fibrinogen, plasminogen, prothrombin, thrombospondin and vitronectin. It recognizes the sequence R-G-D in a wide array of ligands. It recognizes the sequence H-H-L-G-G-G-A-K-Q-A-G-D-V in fibrinogen gamma chain. Following activation integrin alpha-IIb/beta-3 brings about platelet/platelet interaction through binding of soluble fibrinogen. This step leads to rapid platelet aggregation which physically plugs ruptured endothelial cell surface.,polymorphism:Position 874 is associated with platelet-specific alloantigen HPA-3/BAK/LEK. HPA-3A/BAK(A)/LEK(A) has Ile-874 and HPA-3B/BAK(B)/LEK(B) has Ser-874. HPA-3B is involved in neonatal alloimmune thrombocytopenia (NAIT or NATP).,similarity:Belongs to the integrin alpha chain family.,similarity:Contains 7 FG-GAP repeats.,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-IIb associates with beta-3. Directly interacts with RNF181.,tissue specificity:Isoform 1 and isoform 2 were identified in platelets and megakaryocytes, but not in reticulocytes or in Jurkat and U937 white blood cell line. Isoform 3 is expressed by leukemia, prostate adenocarcinoma and melanoma cells but not by platelets or normal prostate or breast epithelial cells.,

Membrane; Single-pass type I membrane protein.

Isoform 1 and isoform 2 are expressed in platelets and megakaryocytes, but not in reticulocytes. Not detected in Jurkat, nor in U937 cell lines (PubMed:2351656). Isoform 3 is expressed in prostate adenocarcinoma, as well as in several erythroleukemia, prostate adenocarcinoma and melanoma cell lines, including PC-3, DU-145, HEL, WM983A, WM983B and WM35. Not detected in platelets, nor in normal prostate (at protein level) (PubMed:9809974).

>>Rap1 signaling pathway ;
>>PI3K-Akt signaling pathway ;
>>Focal adhesion ;
>>ECM-receptor interaction ;
>>Platelet activation ;
>>Neutrophil extracellular trap formation ;
>>Hematopoietic cell lineage ;
>>Regulation of actin cytoskeleton ;
>>Human papillomavirus infection ;
>>Pathways in cancer ;
>>Small cell lung cancer ;
>>Hypertrophic cardiomyopathy ;
>>Arrhythmogenic right ventricular cardiomyopathy ;
>>Dilated cardiomyopathy ;
>>Fluid shear stress and atherosclerosis