ERGIC53 (Biotin)

Catalogue Number: 590574-USB

Manufacturer:United States Biological
Type:Monoclonal Primary Antibody - Conjugated
Shipping Condition:Blue Ice
Unit(s): 100 ug
Host name: Mouse
Clone:
Isotype: IgG1
Immunogen: Synthetic peptide corresponding to 19aa near the C-terminal of human ERGIC53.
Application: ELISA, ICC, WB

Description

Description: 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.

Additional Text

Purification

Affinity Purified

Accession Number

NP_005561

Antibody Clonality

Monoclonal