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LIMP2 Antibody (OAPB00702)

LIMP2 Antibody (OAPB00702)

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

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SPECIFICATIONS

Size

0.1 mg

Applications

ELISA, IHC, WB

Hosts

Rabbit

Reactivities

Hum, Mouse

Presku

OAPB00702

Target

(CT) Lysosomal integral membrane protein 2

Alternative Names

LIMP2 (CT); Lysosomal integral membrane protein 2; CD36L2; Scavenger receptor class B member 2; SR-B2; SCARB2

SUPPLIER INFO

Aviva Systems Biology Incorporated

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