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Purified anti-Laforin Antibody

Pricing & Availability
Clone
N84/37 (See other available formats)
Regulatory Status
RUO
Other Names
Glucan phosphatase, Glycogen phosphatase
Isotype
Mouse IgG1, κ
Ave. Rating
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Product Citations
publications
N84-37_Purified_Laforin_Antibody_1_112118
Western blot of purified anti-Laforin antibody (clone N84/37). Lane 1: Molecular weight marker; Lane 2: 20 µg of human brain membrane lysate. The blot was incubated with 1 µg/mL of the primary antibody overnight at 4°C, followed by incubation with HRP-labeled goat anti-mouse IgG (Cat. No. 405306). Enhanced chemiluminescence were used as the detection system.
  • N84-37_Purified_Laforin_Antibody_1_112118
    Western blot of purified anti-Laforin antibody (clone N84/37). Lane 1: Molecular weight marker; Lane 2: 20 µg of human brain membrane lysate. The blot was incubated with 1 µg/mL of the primary antibody overnight at 4°C, followed by incubation with HRP-labeled goat anti-mouse IgG (Cat. No. 405306). Enhanced chemiluminescence were used as the detection system.
  • N84-37_Purified_Laforin_Antibody_2_112118
    IHC staining of purified anti-Laforin antibody (clone N84/37) on formalin-fixed paraffin-embedded human brain tissue. Following antigen retrieval using Sodium Citrate H.I.E.R, the tissue was incubated with 2.5 µg/mL of the primary antibody overnight at 4°C. BioLegend’s Ultra Streptavidin (USA) HRP Detection Kit (Multi-Species, DAB, Cat. No. 929901) was used for detection followed by hematoxylin (and bluing solution) counterstaining, according to the protocol provided. The image was captured with a 40X objective. Scale bar: 50 µm. Laforin-containing aggresomes are shown in the image.
  • N84-37_Purified_Laforin_Antibody_3_112118
    IHC staining of purified anti-Laforin antibody (clone N84/37) on formalin-fixed paraffin-embedded human skeleton muscle tissue. Following antigen retrieval using Sodium Citrate H.I.E.R, the tissue was incubated with 2.5 µg/mL of the primary antibody overnight at 4°C. BioLegend’s Ultra Streptavidin (USA) HRP Detection Kit (Multi-Species, DAB, Cat. No. 929901) was used for detection followed by hematoxylin (and bluing solution) counterstaining, according to the protocol provided. The image was captured with a 40X objective. Scale bar: 50 µm. Laforin-containing aggresomes are shown in the image.
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858001 25 µg 83 CHF
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858002 100 µg 165 CHF
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Description

Laforin, encoded by the gene EPM2A, is a dual-specificity phosphatase protein. Mutations in EPM2A are the most common mutations found in Lafora disease (LD), a fatal progressive myoclonus epilepsy disease characterized by the accumulation of polyglucosan inclusion bodies, termed Lafora bodies, in various tissues, including neurons, muscle and liver. Through alternative splicing, 2 transcripts are produced from EMP2A gene, a major form termed laf331 and a minor form termed laf317. Laf331 is localized in the cytoplasm while laf317 is targeted to the nucleus. These two isoforms interact with each other and can form homo- and heterodimers.

Malin, encoded by the gene EPM2B, is a ubiquitin ligase also defective in Lafora disease. Inhibition of the ubiquitin proteasome system (UPS) lead to the accumulation of laforin and malin in aggresomes, suggesting that deficits in the UPS system may underlie the pathogenesis of LD. In addition, the laforin/malin complex was shown to negatively regulate glycogen synthesis via modulating cellular glycogen uptake, which is usually high in LD cells. Being a glycogen phosphatase, deficits in laforin also leads to increased phosphorylation of glycogen in vivo, indicating the importance of laforin in glycogen metabolism.

Product Details
Technical Data Sheet (pdf)

Product Details

Verified Reactivity
Human
Antibody Type
Monoclonal
Host Species
Mouse
Immunogen
Fusion protein corresponding to amino acids 1-331 (full-length) of human Laforin
Formulation
Phosphate-buffered solution, pH 7.2, containing 0.09% sodium azide.
Preparation
The antibody was purified by affinity chromatography.
Concentration
0.5 mg/ml
Storage & Handling
The antibody solution should be stored undiluted between 2°C and 8°C.
Application

WB - Quality tested
IHC-P - Verified
Recommended Usage

Each lot of this antibody is quality control tested by Western blotting. For Western blotting, the suggested use of this reagent is 1.0 - 5.0 µg per ml. For immunohistochemistry on formalin-fixed paraffin-embedded tissue, a concentration range of 2.5 - 10 µg/ml is suggested. It is recommended that the reagent be titrated for optimal performance for each application.

RRID
AB_2783481 (BioLegend Cat. No. 858001)
AB_2783482 (BioLegend Cat. No. 858002)

Antigen Details

Structure
Lafora protein is a 331 amino acids tyrosine phosphatase consisting of an N-terminal carbohydrate-binding modue (CBM) and a C-terminal dual-specific phosphatase domain (DSP).
Distribution

Tissue distribution: Laforin is ubiquitously expressed in all tissues, particularly abundant in skeleton muscle, heart, and brain.
Cellular source: Depending on the isoform, this protein can be either in cytosolic or nuclear.

Function
Glycogen metabolism, glycogen dephosphorylation, ubiquitin proteasome system function, protein clearnce
Interaction
Interactions: Malin, AMP-activated protein kinase (AMPK) Cellular targets: the muscle isoform of glycogen synthase, PTG, protein phosphatase type 1 (PP1), neuronatin
Ligand/Receptor
Laforin was shown to be regulated by the glycogen storage in cell, but the mechanism is unclear.
Biology Area
Cell Biology, Neurodegeneration, Neuroscience, Protein Trafficking and Clearance
Molecular Family
Protein Kinases/Phosphatase, Enzymes and Regulators
Antigen References
  1. Gentry MS, et al. 2013. FEBS J. 280(2):525-37.
  2. Sankhala RS, et al. 2015. J Biol chem. 290(8):4552-9.
  3. Singh PK, et al. 2012. Mol Cell Biol. 32(3):652-63.
  4. Mittal S, et al. 2007. Hum Mol Genet. 16(7):753-62.
  5. Tagliabracci VS, et al. 2007. Proc. Natl. Acad. Sci U S A. 104(49):19262-6.
Gene ID
7957 View all products for this Gene ID
UniProt
View information about Laforin on UniProt.org

Related FAQs

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Go To Top Version: 1    Revision Date: 11.21.2018

For Research Use Only. Not for diagnostic or therapeutic use.

 

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