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LIMP2 Antibody

Catalog # Availability Size / Price Inquiry
APR08226G 0.1 mg / $695

LIMP2 Antibody

Brand

Leading Biology

Catalog Number

APR08226G

Product Type

Polyclonal Antibodies

Field of Research

Product Overview

We constantly strive to ensure we provide our customers with the best antibodies. As a result of this work we offer this antibody in purified format. We are in the process of updating our datasheets. If you have any questions regarding this update, please feel free to contact our technical support team. This product is a high quality LIMP2 antibody.

Molecular Weight

Predicted: 53 kDa Observed: 57 kDa

Cellular Localization

Antigen Cellular Localization: Lysosome membrane; Multi-pass membrane protein

Host

Rabbit

Species Reactivity

Human, Mouse, Rat

Target

SCARB2;

Isotype

IgG

Symbol

CD36L2, LIMP2, LIMPII

GeneID

UniProt ID

Function

Acts as a lysosomal receptor for glucosylceramidase (GBA) targeting.

Summary

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.

Form

Liquid

Storage & Stability

Store at +4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.

Applications

WB, IHC-P, IF, E

Images

Western blot analysis of LIMP2 in mouse liver tissue lysate with LIMP2 antibody at 1 μg/mL in (A) the absence and (B) presence of blocking peptide.

Immunohistochemistry of LIMP2 in human skeletal muscle tissue with LIMP2 antibody at 2.5 μg/mL.

Immunofluorescence of LIMP2 in Human Skeletal Muscle tissue with LIMP2 antibody at 20 μg/mL.

Specification

Quantity

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