Product Name:
Laforin
Product Number:
ab-nn245-1
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: Laforin
Antibody Type: Monoclonal
Antibody Host Species: Mouse
Antibody Ig Isotype Clone: IgG1
Antibody Immunogen Source: Fusion protein with full-length of human Laforin
Antibody Immunogen Description: Corresponds to amino acid residues M1-L331. 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: This antibody detects the target protein in the following species due to conservation of amino acid sequence: 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 that is encoded by the EPM2A gene. It acts as a crucial glycogen phosphatase, preventing the formation of insoluble, neurotoxic glycogen deposits known as Lafora bodies. These are poorly branched glycogen molecules (polyglucosans). Laforin features a unique combination of a C-terminal dual-specificity phosphatase (DSP) domain and an N-terminal carbohydrate-binding module (CBM20), making it a member of the glucan phosphatase family. 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), specifically through the E3-ubiquitin ligase malin. Although it is expressed throughout the body, it is most abundant in tissues with high glycogen metabolism, including the brain, skeletal muscle, heart, and liver, as well as kidney and pancreas. Laforin is expressed in heart, skeletal muscle, kidney, pancreas and brain. Defective Laforin is linked to progressive myoclonic epilepsy type 2 (EPM2), also known as Lafora disease. This description may include information annotated by UniProt and/or Google AI.