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EPM2A / Laforin Antibody (aa80-130)

Rabbit Polyclonal Antibody

     
  • WB - EPM2A / Laforin Antibody (aa80-130) ALS15246
    Western blot of EPM2A in human heart lysate in the 1) absence and2) presence of immunizing...
    detail
  • IHC - EPM2A / Laforin Antibody (aa80-130) ALS15246
    Anti-EPM2A / Laforin antibody IHC of human heart.
    detail
  • SPECIFICATION
  • CITATIONS
  • PROTOCOLS
  • BACKGROUND
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Product Information
Application
  • Applications Legend:
  • WB=Western Blot
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin-embedded Sections)
  • IHC-F=Immunohistochemistry (Frozen Sections)
  • IF=Immunofluorescence
  • FC=Flow Cytopmetry
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • E=ELISA
  • IP=Immunoprecipitation
  • DB=Dot Blot
  • CHIP=Chromatin Immunoprecipitation
  • FA=Fluorescence Assay
  • IEM=Immunoelectronmicroscopy
  • EIA=Enzyme Immunoassay
WB, IHC-P
Primary Accession O95278
Reactivity Human, Bovine
Host Rabbit
Clonality Polyclonal
Calculated MW 37kDa
Dilution IHC-P (10 µg/ml), WB (0.25-0.5 µg/ml),
Additional Information
Gene ID 7957
Other Names Laforin, 3.1.3.-, 3.1.3.16, 3.1.3.48, Glucan phosphatase, Lafora PTPase, LAFPTPase, EPM2A
Target/Specificity Human EPM2A
Reconstitution & Storage Store at 4°C for short term applications. For long term storage, aliquot and store at -20°C.
PrecautionsEPM2A / Laforin Antibody (aa80-130) is for research use only and not for use in diagnostic or therapeutic procedures.
Protein Information
Name EPM2A
Function 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).
Cellular Location Cytoplasm. Note=Under glycogenolytic conditions localizes to the nucleus [Isoform 2]: Cytoplasm. Endoplasmic reticulum membrane; Peripheral membrane protein; Cytoplasmic side. Cell membrane. Nucleus. Note=Also found in the nucleus. [Isoform 5]: Cytoplasm. Nucleus
Tissue Location Expressed in heart, skeletal muscle, kidney, pancreas and brain. Isoform 4 is also expressed in the placenta
Research Areas
Citations (0)
citation

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Background

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.

References

Minassian B.A.,et al.Nat. Genet. 20:171-174(1998).
Ganesh S.,et al.Hum. Mol. Genet. 9:2251-2261(2000).
Lee J.R.,et al.Submitted (AUG-1998) to the EMBL/GenBank/DDBJ databases.
Ganesh S.,et al.Submitted (NOV-2001) to the EMBL/GenBank/DDBJ databases.
Ota T.,et al.Nat. Genet. 36:40-45(2004).

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Discontinued
Cat# ALS15246
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