GTX85033 IHC-P Image
LIMP II antibody
GTX85033
ApplicationsWestern Blot, ELISA, ImmunoHistoChemistry, ImmunoHistoChemistry Paraffin
Product group Antibodies
ReactivityHuman, Mouse, Rat
Overview
- SupplierGeneTex
- Product NameLIMP II antibody
- Delivery Days Customer10
- ApplicationsWestern Blot, ELISA, ImmunoHistoChemistry, ImmunoHistoChemistry Paraffin
- CertificationResearch Use Only
- ClonalityPolyclonal
- Concentration1 mg/ml
- HostRabbit
- IsotypeIgG
- Scientific DescriptionThe 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.
- ReactivityHuman, Mouse, Rat
- Storage Instruction2°C to 8°C,-20°C
- UNSPSC12352203