Application
| WB, ICC |
---|---|
Primary Accession | O95278 |
Other Accession | NP_001018051.1 |
Host | Mouse |
Isotype | IgG1 |
Reactivity | Human |
Clonality | Monoclonal |
Description | Mouse Anti-Human Laforin Monoclonal IgG1 |
Target/Specificity | Detects ~40kDa. |
Other Names | EPM2 Antibody, Epilepsy progressive myoclonus type 2 Lafora disease (laforin) Antibody, Epilepsy progressive myoclonus type 2A Lafora disease (laforin) Antibody, EPM2 Antibody, Epm2a Antibody, Lafora PTPase Antibody, LAFPTPase Antibody, LD Antibody, LDE Antibody, MELF Antibody |
Clone Names | S84-37 |
Immunogen | Fusion protein amino acids 1-331 (full-length) of human Laforin. Rat: 90% identity (296/327 amino acids identical). Mouse: 89% identity (295/327 amino acids identical) |
Purification | Protein G Purified |
Storage | -20ºC |
Storage Buffer | PBS pH 7.4, 50% glycerol, 0.1% sodium azide |
Shipping Temperature | Blue Ice or 4ºC |
Certificate of Analysis | 1 µg/ml of SMC-466 was sufficient for detection of Laforin in 20 µg of human brain lysate by colorimetric immunoblot analysis using Goat anti-mouse IgG:HRP as the secondary antibody. |
Cellular Localization | Endoplasmic Reticulum |
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Provided below are standard protocols that you may find useful for product applications.
Background
Laforin, also known as Lafora PTPase, is a dual specificity protein phosphatase. Laforin is involved in the control of glycogen metabolism, specifically in preventing the formation of poorly branched glycogen molecules (polyglucosans). Laforin forms a complex with NHLRC1/malin and HSP70 that suppresses the cellular toxicity of misfolded proteins by promoting their degradation through the ubiquitin-proteasome system (UPS). Laforin is expressed in heart, skeletal muscle, kidney, pancreas and brain. Defective Laforin is linked to progressive myoclonic epilepsy type 2 (EPM2).