LIMP2 Antibody
Product Details
- Cat. No.
- 253882
- Type
- Primary Antibody
- Clonality
- Polyclonal
- Host
- Rabbit
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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.
Biological Information
- Clonality: Polyclonal
- Host: Rabbit
- Reactivity: Human, Mouse, Rat
- Antigen: KLH-conjugated synthetic peptide encompassing a sequence within human LIMP2.
- Isotype: Rabbit Ig
- Gene: AAH21892
Handling
- Quantity: 0.1 mg
- Storage: Store at -20°C. Minimize freeze-thaw cycles. Product is guaranteed one year from the date of shipment.
Applications
- ELISA (ELISA)
- Immunohistochemistry (IHC)
- Western Blotting (WB)