SCARB2, CT (Lysosome Membrane Protein 2, 85 kDa Lysosomal Membrane Sialoglycoprotein, LGP85, CD36 Antigen-like 2, Lysosome Membrane Protein II, LIMP II, Scavenger Receptor Class B Member 2, CD36, CD36L2, LIMPII)

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 20aa. 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 b-glucocerebrosidase, the enzyme defective in Gaucher disease, a lysosomal storage disorder. LIMP2-deficient mice showed missorted as well as secreted b-glucocerebrosidase, suggesting that LIMP2 also functions as the mannose-6-phosphate-independent trafficking receptor. Despite its predicted molecular weight, LIMP2 runs at approximately 80-85kD in SDS-PAGE.\n\nApplications:\nSuitable for use in ELISA, Western Blot and Immunohistochemistry. Other applications not tested.\n\nRecommended Dilution:\nWestern Blot: 1-2ug/ml\nImmunohistochemistry (Formalin fixed paraffin embedded): 10ug/ml\nOptimal dilutions to be determined by the researcher.\n\nPositive Control:\nHuman Skeletal Muscle Tissue Lysate\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

146300

Size

100ug

Applications

ELISA, IHC, WB

Hosts

Rabbit

Reactivities

Hum, Mouse

Form

Supplied as a liquid in PBS, 0.02% sodium azide.

P Type

Pab

Purity

Purified by immunoaffinity chromatography.

Isotype

IgG

References

1. Fujita H, Saeki M, Yasunaga K, et al. Isolation and sequencing of a cDNA clone encoding 85kDa sialoglycoprotein in rat liver lysosomal membranes. Biochem. Biophys. Res. Commun. 1991; 178:444-52.\n2. Gamp A, Tanaka Y, Lullmann-Rauch R, et al. LIMP-2/LGP85 deficiency causes uretic pelvic junction obstruction, deafness and peripheral neuropathy in mice. Hum. Mol. Genet. 2003; 12:631-46. 3. Knipper M, Claussen C, Ruttiger L, et al. Deafness in LIMP2-deficient mice due to early loss of the potassium channel KCNQ1/KCNE1 in marginal cells of the stria vascularis. J. Physiol. 2006; 576:73-86. 4. Reczek D, Schwake M, Schroder J, et al. LIMP-2 is a receptor for lysosomal mannose-6-phosphate-independent targeting of b-glucocerebrosidase. Cell 2007; 131:770-83.

Additional Info

Recognizes human LIMP2. Species Crossreactivity: mouse.