LIMP2 Antibody

LIMP2 Antibody

Catalog# :4621

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 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 – 85 kDa in SDS-PAGE.
Additional Names : LIMP2, Lysosomal integral membrane protein 2, CD36L2, Scavenger receptor class B member 2, SR-B2, SCARB2
Description
Left: Western blot analysis of LIMP2 in human skeletal muscle tissue lysate with LIMP2 antibody at 1 µg/ml in (A) the absence and (B) presence of blocking peptide.

Below: Immunohistochemistry of LIMP2 in human skeletal muscle with LIMP2 antibody at 2.5 µg/ml.

Other Product Images

Source :LIMP2 antibody was raised against a 16 amino acid peptide from near the center of human LIMP2.
Purification : Affinity chromatography purified via peptide column
Clonality and Clone : This is a polyclonal antibody.
Host : LIMP2 antibody was raised in rabbit. Please use anti-rabbit secondary antibodies.
Application : LIMP2 antibody can be used for detection of LIMP2 by Western blot at 1 – 2 µg/ml.
Tested Application(s) : E, WB, IHC
Buffer : Antibody is supplied in PBS containing 0.02% sodium azide.
Blocking Peptide :Cat.No. 4621P - LIMP2 Peptide
Long-Term Storage : LIMP2 antibody can be stored at 4ºC, stable for 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.
Positive Control :
1. Cat. No. 1375 - Human Skeletal Muscle Tissue Lysate
Species Reactivity : H, M, R
GI Number : 18257312
Accession Number : AAH21892
Short Description : Lysosomal integral membrane protein 2
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.
2. 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.