Anti-Laforin EPM2A Antibody

The Epilepsy, progressive myoclonus type 2A protein (EPM2A) is a dual-specificity phosphatase that associates with polyribosomes. Mutations in this gene have been associated with myoclonic epilepsy of Lafora. EPM2A interacts with a number of proteins known to be involved in glycogen metabolism and has been shown to have robust phosphatase activity against a phosphorylated complex carbohydrate, suggesting that EPM2A may be involved in the regulation of glycogen metabolism.

EPM2A antibody was raised against a 17 amino acid synthetic peptide near the carboxy terminus of human EPM2A. The immunogen is located within amino acids 190 - 240 of EPM2A.

Plays an important role in preventing glycogen hyperphosphorylation and the formation of insoluble aggregates, via its activity as glycogen phosphatase, and by promoting the ubiquitination of proteins involved in glycogen metabolism via its interaction with the E3 ubiquitin ligase NHLRC1/malin. Shows strong phosphatase activity towards complex carbohydrates in vitro, avoiding glycogen hyperphosphorylation which is associated with reduced branching and formation of insoluble aggregates (PubMed:16901901, PubMed:23922729, PubMed:26231210, PubMed:25538239, PubMed:25544560) . Dephosphorylates phosphotyrosine and synthetic substrates, such as para- nitrophenylphosphate (pNPP), and has low activity with phosphoserine and phosphothreonine substrates (in vitro) (PubMed:11001928, PubMed:11220751, PubMed:11739371, PubMed:14532330, PubMed:16971387, PubMed:18617530, PubMed:22036712, PubMed:23922729, PubMed:14722920) . Has been shown to dephosphorylate MAPT (By similarity) . Forms a complex with NHLRC1/malin and HSP70, which suppresses the cellular toxicity of misfolded proteins by promoting their degradation through the ubiquitin-proteasome system (UPS) . Acts as a scaffold protein to facilitate PPP1R3C/PTG ubiquitination by NHLRC1/malin (PubMed:23922729) . Also promotes proteasome-independent protein degradation through the macroautophagy pathway (PubMed:20453062) .

EPM2A antibody can be stored at 4°C for three months and -20°C, stable for up to one year. Avoid repeated freeze-thaw cycles. Antibodies should not be exposed to prolonged high temperatures.