LIMP2 Antibody

LIMP2 Antibody: 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.

LIMP2 antibody can be stored at 4˚ C for three months and -20˚ C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

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.

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.

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.

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.

LIMP2 Antibody: AMRF, EPM4, LGP85, CD36L2, HLGP85, LIMP-2, LIMPII, SR-BII, LIMP2, Lysosome membrane protein 2, 85 kDa lysosomal membrane sialoglycoprotein