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Product
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LIMP2 Antibody
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Cat#
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100-11-65A
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Unit/Weight
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100 μg
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Unit Price
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$311.00
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Species Reactivity
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H, M
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Tested Application(s)
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E, WB
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Brief Description
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(CT) Lysosomal integral membrane protein 2
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Specific Description
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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.
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Storage
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LIMP2 antibody can be stored at 4°C, 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.
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