| Host: | Rabbit |
| Applications: | IHC/WB |
| Reactivity: | Human |
| Note: | STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS. |
| Short Description: | Rabbit polyclonal antibody anti-Laforin (50-100 aa) is suitable for use in Immunohistochemistry and Western Blot research applications. |
| Clonality: | Polyclonal |
| Conjugation: | Unconjugated |
| Isotype: | IgG |
| Formulation: | Lyophilised |
| Purification: | IgG purified |
| Dilution Range: | IHC, WB (confirmed by recombinant protein). A dilution of 1:300 to 1:2000 is recommended. The optimal dilution should be determined by the end user. Not yet tested in other applications. |
| Storage Instruction: | Maintain the lyophilised/reconstituted antibodies frozen at-20°C for long term storage and refrigerated at 2-8°C for a shorter term. When reconstituting, Glycerol (1:1) may be added for an additional stability. Avoid freeze and thaw cycles. |
| Gene Symbol: | EPM2A |
| Gene ID: | 7957 |
| Uniprot ID: | EPM2A_HUMAN |
| Immunogen Region: | 50-100 aa |
| Specificity: | Specific for Laforin. |
| Immunogen: | A synthetic peptide from aa region 50-100 of mouse Laforin conjugated to an immunogenic carrier protein was used as the antigen. The antigen is homologous in human. |
| 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. |
| 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. Dephosphorylates phosphotyrosine and synthetic substrates, such as para-nitrophenylphosphate (pNPP), and has low activity with phosphoserine and phosphothreonine substrates (in vitro). Has been shown to dephosphorylate MAPT. 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: Does not bind to glycogen. Lacks phosphatase activity and might function as a dominant-negative regulator for the phosphatase activity of isoform 1 and isoform 7. Isoform 7: Has phosphatase activity (in vitro). |
| Protein Name | Laforin Glucan Phosphatase Glycogen Phosphatase Lafora Ptpase Lafptpase |
| Database Links | Reactome: R-HSA-3322077 Reactome: R-HSA-3785653 |
| Cellular Localisation | Cytoplasm Under Glycogenolytic Conditions Localizes To The Nucleus Isoform 1: Cytoplasm Endoplasmic Reticulum Membrane Peripheral Membrane Protein Cytoplasmic Side Cell Membrane Colocalizes With Glycogen Synthase In Punctate Structures In The Cytoplasm Primarily Associated With Polyribosomes At The Rough Endoplasmic Reticulum And Also Detected At The Plasma Membrane Isoform 2: Cytoplasm Nucleus Also Found In The Nucleus Isoform 4: Cytoplasm Isoform 5: Cytoplasm Isoform 7: Cytoplasm |
| Alternative Antibody Names | Anti-Laforin antibody Anti-Glucan Phosphatase antibody Anti-Glycogen Phosphatase antibody Anti-Lafora Ptpase antibody Anti-Lafptpase antibody Anti-EPM2A antibody |
Information sourced from Uniprot.org
