别名：ERGIC-53; ERGIC53; F5F8D; FMFD1; gp58; MCFD1; MR60

产品概述：Carrier-free (BSA/glycerol-free) LMAN1 mouse monoclonal antibody, clone LBI1A8

总结：The protein encoded by this gene 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. [provided by RefSeq]