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Rabbit Anti-EPM2A Antibody (CSB-PA007738ESR1HU)
Supplier: CUSABIO BIOTECH CO.
Glucan phosphatase Antibody,Lafora PTPase Antibody,LAFPTPase Antibody,EPM2A AntibodyMore alternative names for the antibody
Epilepsy progressive myoclonus type 2 Lafora disease (laforin) antibody|Epilepsy progressive myoclonus type 2A Lafora disease (laforin) antibody|EPM2 antibody|Epm2a antibody|Epm2a gene antibody|EPM2A_HUMAN antibody|Lafora PTPase antibody|Laforin antibody|LAFPTPase antibody|LD antibody|LDE antibody|MELF antibody|RP3-466P17.2 antibody
Anti-EPM2A antibody (ab129321)
Anti-EPM2A antibody (ab129321)
Recommended applications: ELISA, IHC
Recommended dilution: Recommended dilution:IHC:1:20-1:200
Recommended protocols: check protocols
|Catalogue No.|| |
Recombinant human Laforin protein (244-331AA)
|Recommended dilution|| |
|Molecular weight|| |
Antigen Affinity Purified
Shipped at 4 Celcius Degree. Upon delivery aliquot and store at -20 Celcius Degree or -80 Celcius Degree. Avoid repeated freeze.
|Database links|| |
Entrez Gene:484022( Dog), Entrez Gene:7957( Human), Entrez Gene:13853( Mouse), Entrez Gene:114005( Rat), Omim:607566( Human), SwissProt:Q1M199( Dog), SwissProt:O95278( Human), SwissProt:Q9WUA5( Mouse), SwissProt:Q91XQ2( Rat), Unigene:486696( Human), Unigene:89946( Mouse), Unigene:121954( Rat)
|Protein function|| |
Has both dual-specificity protein phosphatase and glucan phosphatase activities. Together with the E3 ubiquitin ligase NHLRC1/malin, appears to be involved in the clearance of toxic polyglucosan and protein aggregates via multiple pathways. Dephosphorylates phosphotyrosine, phosphoserine and phosphothreonine substrates in vitro. Has also been shown to dephosphorylate MAPT. Shows strong phosphatase activity towards complex carbohydrates in vitro, avoiding glycogen hyperphosphorylation which is associated with reduced branching and formation of insoluble aggregates. 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. Also promotes proteasome-independent protein degradation through the macroautophagy pathway. Isoform 2, an inactive phosphatase, could function as a dominant-negative regulator for the phosphatase activity of isoform 1. .
|Protein tissue specificity|| |
Expressed in heart, skeletal muscle, kidney, pancreas and brain. Isoform 4 is also expressed in the placenta. .
|Involvement in disease|| |
Epilepsy, progressive myoclonic 2 (EPM2) [MIM:254780]: An autosomal recessive and severe form of adolescent-onset progressive epilepsy. Typically, as seizures increase in frequency, cognitive function declines towards dementia, and affected individuals die usually within 10 years after onset. EPM2 occurs worldwide, but it is particularly common in the mediterranean countries of southern Europe and northern Africa, in southern India and in the Middle East. At the cellular level, it is characterized by accumulation of starch-like polyglucosans called Lafora bodies (LBs) that are most abundant in organs with the highest glucose metabolism: brain, heart, liver and skeletal muscle. Among other conditions involving polyglucosans, EPM2 is unique in that the inclusions are in neuronal dendrites but not axons and the forming polyglucosan fibrils are associated with the endoplasmic reticulum. . Note=The disease is caused by mutations affecting the gene represented in this entry.
|Protein sequence and domain|| |
Belongs to the protein-tyrosine phosphatase family.
|Protein post-translational modifications|| |
Polyubiquitinated by NHLRC1/malin. .; Phosphorylation on Ser-25 by AMPK affects the phosphatase activity of the enzyme and its ability to homodimerize and interact with NHLRC1, PPP1R3C or PRKAA2. .
|Protein cellular localization|| |
Cytoplasm . Note=Under glycogenolytic conditions localizes to the nucleus.; Isoform 1: Endoplasmic reticulum. Cell membrane. Note=Primarily associated with polyribosomes at the endoplasmic reticulum, also found at the plasma membrane.; Isoform 2: Endoplasmic reticulum . Cell membrane . Nucleus . Note=Also found in the nucleus.; Isoform 4: Cytoplasm. Nucleus.; Isoform 5: Cytoplasm. Nucleus.; Isoform 7: Cytoplasm.
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CUSABIO BIOTECH CO.
|Product type|| |
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