Product Name: Laforin
Product Number: AB-NN245-1
Size: | 25 µg | | Price: | 89.00 |
| | | $US | |
Target Full Name: Epilepsy progressive myoclonus type 2 Lafora disease
Target Alias: EPM2; Epilepsy progressive myoclonus type 2A Lafora disease (laforin); EPM2; Epm2a; Lafora PTPase; LAFPTPase; LD; LDE; MELF
Product Type Specific: Laforin pan-specific antibody
Antibody Code: NN245-1
Antibody Target Type: Pan-specific
Protein UniProt: O95278 Protein SigNET: O95278 Antibody Type: Monoclonal
Antibody Host Species: Mouse
Antibody Ig Isotype Clone: IgG1
Antibody Immunogen Source: 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)
Production Method: Protein G purified
Antibody Modification: Unconjugated. Contact KInexus if you are interest in having the antibody biotinylated or coupled with fluorescent dyes.
Antibody Concentration: 1 mg/ml
Storage Buffer: Phosphate buffered saline pH 7.4, 50% glycerol, 0.1% sodium azide
Storage Conditions: For long term storage, keep frozen at -40°C or lower. Stock solution can be kept at +4°C for more than 3 months. Avoid repeated freeze-thaw cycles.
Product Use: Western blotting | ICC/Immunofluorescence
Antibody Dilution Recommended: WB (1:1000); optimal dilutions for assays should be determined by the user.
Antibody Potency: In mouse brain lysates, this antibody detects a ~40 kDa protein by Western blotting.
Antibody Species Reactivity: Human
Antibody Positive Control: 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.
Antibody Specificity: Very high
Antibody Cross Reactivity: One weak ~70 kDa cross-reactive protein and possibly a very weak ~40 kDa target protein in mouse brain lysates.
Scientific 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).