LIMPII/SR-B2 Antibody - BSA Free

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.

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

We have publications tested in 1 confirmed species: Rat.

We have publications tested in 1 application: IHC-Fr.

Showing Publications 1 - 5 of 5.

Publications using NBP1-77326	Applications	Species
Roet KC, Franssen EH, de Bree FM et al. A Multilevel Screening Strategy Defines a Molecular Fingerprint of Proregenerative Olfactory Ensheathing Cells and Identifies SCARB2, a Protein That Improves Regenerative Sprouting of Injured Sensory Spinal Axons. J Neurosci 2013-07-03 [PMID: 23825416] (IHC-Fr, Rat)	IHC-Fr	Rat
Reczek D, Schwake M, Schroder J et al. LIMP-2 is a receptor for lysosomal mannose-6-phosphate-independent targeting of b-glucocerebrosidase. Cell; 131:770-83.]. 2007-01-01 [PMID: 18022370]
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. 576:73-86. 2006-01-01 [PMID: 16901941]
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. 12:631-46. 2003-01-01 [PMID: 12620969]
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. 178:444-52. 1991-01-01 [PMID: 1859403]

Images	Ratings	Applications	Species	Date	Details
Enlarge	3 reviewed by: Verified Customer	Simple Western	Human and Rat	10/31/2018	View Summary Application Simple Western Sample Tested B103 whole cell lysate,SVG-A whole cell lysate Species Human and Rat Lot 4621-1003

Array NBP1-77326

• LIMPII/SR-B2 Antibodies
• LIMPII/SR-B2 Lysate
• LIMPII/SR-B2 Proteins
• LIMPII/SR-B2 Proteins and Enzymes