LIMP2 Antibody

Catalog Number:

24706

Amount:

100μg

Price:

$319

Swiss-Prot No:

Swiss-Prot:Q14108
Gene ID:950

Form of Antibody:

Supplied in PBS containing 0.02% sodium azide.

Storage/Stability:

Immunogen:

Raised against a 18 amino acid peptide from near the carboxy terminus of human LIMP2.

Purification:

Affinity chromatography purified via peptide column

Specificity/Sensitivity:

Applications:

ELISA WB IHC

Background:

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.

References:

appl_detail:

Datasheet PDF