ERGIC53 Monoclonal Antibody: ERGIC53, also known as lectin mannose-binding 1 (LMAN1) protein is a type I integral membrane protein localized in the intermediate region between the endoplasmic reticulum and the Golgi, presumably recycling between the two compartments. The protein is a mannose-specific lectin and is a member of a novel family of plant lectin homologs in the secretory pathway of animal cells. Mutations in the gene are associated with a coagulation defect. Using positional cloning, the gene was identified as the disease gene leading to combined factor V-factor VIII deficiency, a rare, autosomal recessive disorder in which both coagulation factors V and VIII are diminished.
应用类型
E, WB, ICC
免疫原
Mouse monoclonal ERGIC53 antibody was raised against a 19 amino acid peptide near the carboxy terminus of human ERGIC53.
来源宿主
小鼠
反应性
人, 小鼠
保存建议
ERGIC53 Monoclonal antibody can be stored at 4˚C for three months and -20˚C, stable for up to one year.