LIMP2 polyclonal, anti-human, mouse

Protein Family: Enzymes

Pathway and Disease: Signaling Molecules and Interaction

Description:
The lysosomal integral membrane protein 2 (LIMP2) is a heavily glycosylated type III transmembrane protein, the majority of which exists in the lumen of the lysosome and a cytoplasmic domain of approximately 20 amino acids. A deficiency of LIMP2 in mice causes uretic pelvic junction obstruction, deafness, and peripheral neuropathy associated with impaired vesicular trafficking and distribution of apically expressed proteins. More recently, LIMP2 was shown to act as a receptor to bind beta-glucocerebrosidase, the enzyme defective in Gaucher disease, a lysosomal storage disorder. LIMP2-deficient mice showed missorted as well as secreted beta-glucocerebrosidase, suggesting that LIMP2 also functions as the mannose-6-phosphate-independent trafficking receptor. Despite its predicted molecular weight, LIMP2 runs at approximately 80 – 85 kDa in SDS-PAGE.

Alternate Names: LIMP2 , Lysosomal integral membrane protein 2, CD36L2, Scavenger receptor class B member 2, SR-B2, SCARB2

Application Notes: E: 1:500-1:1,000; WB: 1:100-1:500; IHC: 1:100-1:500

Accession No.: AAH21892

Antigen: KLH-conjugated synthetic peptide encompassing a sequence within human LIMP2.

Format: Each vial contains 0.1 ml IgG in PBS pH 7.4 with 0.02% sodium azide. Antibody was purified by immunogen affinity chromatography.

Storage:
Store at -20°C. Minimize freeze-thaw cycles. Product is guaranteed one year from the date of shipment.