- N84/37 (See other available formats)
- Other Names
- Glucan phosphatase, Glycogen phosphatase
- Mouse IgG1, κ
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- Product Citations
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Laforin, encoded by the gene EPM2A, is a dual-specificity phosphatase protein. Mutations in EPM2A are the most common mutations found in Lafora disease (LD), a fatal progressive myoclonus epilepsy disease characterized by the accumulation of polyglucosan inclusion bodies, termed Lafora bodies, in various tissues, including neurons, muscle and liver. Through alternative splicing, 2 transcripts are produced from EMP2A gene, a major form termed laf331 and a minor form termed laf317. Laf331 is localized in the cytoplasm while laf317 is targeted to the nucleus. These two isoforms interact with each other and can form homo- and heterodimers.
Malin, encoded by the gene EPM2B, is a ubiquitin ligase also defective in Lafora disease. Inhibition of the ubiquitin proteasome system (UPS) lead to the accumulation of laforin and malin in aggresomes, suggesting that deficits in the UPS system may underlie the pathogenesis of LD. In addition, the laforin/malin complex was shown to negatively regulate glycogen synthesis via modulating cellular glycogen uptake, which is usually high in LD cells. Being a glycogen phosphatase, deficits in laforin also leads to increased phosphorylation of glycogen in vivo, indicating the importance of laforin in glycogen metabolism.
- Antibody Type
- Host Species
- Fusion protein corresponding to amino acids 1-331 (full-length) of human Laforin
- Phosphate-buffered solution, pH 7.2, containing 0.09% sodium azide.
- The antibody was purified by affinity chromatography.
- 0.5 mg/ml
- Storage & Handling
- The antibody solution should be stored undiluted between 2°C and 8°C.
WB - Quality tested
IHC-P - Validated
- Recommended Usage
Each lot of this antibody is quality control tested by Western blotting. For Western blotting, the suggested use of this reagent is 1.0 - 5.0 µg per ml. For immunohistochemistry on formalin-fixed paraffin-embedded tissue, a concentration range of 2.5 - 10 µg/ml is suggested. It is recommended that the reagent be titrated for optimal performance for each application.
AB_2783481 (BioLegend Cat. No. 858001)
AB_2783482 (BioLegend Cat. No. 858002)
- Lafora protein is a 331 amino acids tyrosine phosphatase consisting of an N-terminal carbohydrate-binding modue (CBM) and a C-terminal dual-specific phosphatase domain (DSP).
Tissue distribution: Laforin is ubiquitously expressed in all tissues, particularly abundant in skeleton muscle, heart, and brain.
Cellular source: Depending on the isoform, this protein can be either in cytosolic or nuclear.
- Glycogen metabolism, glycogen dephosphorylation, ubiquitin proteasome system function, protein clearnce
- Interactions: Malin, AMP-activated protein kinase (AMPK) Cellular targets: the muscle isoform of glycogen synthase, PTG, protein phosphatase type 1 (PP1), neuronatin
- Ligand Receptor
- Laforin was shown to be regulated by the glycogen storage in cell, but the mechanism is unclear.
- Biology Area
- Cell Biology, Neurodegeneration, Neuroscience, Protein Trafficking and Clearance
- Molecular Family
- Protein Kinases/Phosphatase, Enzymes and Regulators
- Antigen References
- Gentry MS, et al. 2013. FEBS J. 280(2):525-37.
- Sankhala RS, et al. 2015. J Biol chem. 290(8):4552-9.
- Singh PK, et al. 2012. Mol Cell Biol. 32(3):652-63.
- Mittal S, et al. 2007. Hum Mol Genet. 16(7):753-62.
- Tagliabracci VS, et al. 2007. Proc. Natl. Acad. Sci U S A. 104(49):19262-6.
- Gene ID
- 7957 View all products for this Gene ID
- View information about Laforin on UniProt.org
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