Rabbit polyclonal antibody to LIMP IIThe 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.
Key Feature
Clonality
Polyclonal
Host Species
Rabbit
Tested Applications
ELISAIHC-PWB
IHC-P:1:50~1:200 WB:1:500~1:2000 Notes:Optimal dilutions/concentrations should be determined by the researcher.
Species Reactivity
HumanMouse
Concentration
1mg/ml
Purification
Affinity purified
Target Information
Gene Synonyms
CD36L2 Lysosomal integral membrane protein 2 SR-B2 SRB2 Scavenger receptor class B member2 Lysosomal integral membrane protein2 SCARB2 LIMP2 Scavenger receptor class B member 2 SR B2
Target
LIMP2
Tissue Specificity
This antibody is specific for LIMP2
Application
Application
Immunohistochemistry of LIMP2 in human skeletal muscle tissue with LIMP2 antibody at 10 ug/ml.
Application
Western blot analysis of LIMP2 in human skeletal muscle tissue lysate with LIMP2 antibody at (A) 1 and (B) 2 ug/ml.