• Biotinylated STJ71586 (1µg/ml) staining of Human Cerebellum lysate (35µg protein in RIPA buffer) , exactly mirroring its parental non-biotinylated product. Primary incubation was 1 hour. Detected by chemiluminescence, using streptavidin-HRP and using NAP blocker as a substitute for skimmed milk.

Anti-Laforin, Biotinylated-Isoform a antibody (Internal) {Biotin} (STJ73180)

SKU:
STJ73180-100

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Host: Goat
Applications: Pep-ELISA/WB/IHC
Reactivity: Human/Mouse/Rat/Dog/Cow
Note: STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS.
Short Description: Goat polyclonal antibody anti-Laforin, Biotinylated-Isoform a (Internal) is suitable for use in ELISA, Western Blot and Immunohistochemistry research applications.
Clonality: Polyclonal
Conjugation: Biotin
Isotype: IgG
Formulation: 0.5 mg/ml in Tris saline, 0.02% sodium azide, pH7.3 with 0.5% bovine serum albumin. NA
Purification: Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
Concentration: 0.5 mg/mL
Dilution Range: WB-1-3µg/ml
ELISA-antibody detection limit dilution 1:16000.
Storage Instruction: Store at-20°C on receipt and minimise freeze-thaw cycles.
Gene Symbol: EPM2A
Gene ID: 7957
Uniprot ID: EPM2A_HUMAN
Immunogen Region: Internal
Accession Number: NP_005661.1
Specificity: This antibody is expected to recognize isoform a (NP_005661.1) only.
Immunogen Sequence: EATGHTNEMKHTTD.
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

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