EPM2A Antibody

Size

100ul

Clone Number

Aliases

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

Immunogen Species

Homo sapiens (Human)

UniProt ID

O95278

Immunogen

Recombinant Human Laforin protein (244-331AA)

Raised in

Rabbit

Species Reactivity

Human

Tested Applications

ELISA, IHC; Recommended dilution: IHC:1:20-1:200

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.

Clonality

Polyclonal

Isotype

IgG

Purification Method

Antigen Affinity Purified

Conjugate

Non-conjugated

Buffer

PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Form

Liquid

Stroage

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Target Names

EPM2A

Research Areas

Metabolism; Signal transduction