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anti-EPM2A antibody :: Goat EPM2A / Laforin Polyclonal Antibody

Scan QR to view Datasheet Catalog #    MBS243650
Unit / Price
0.05 mg  /  $495 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

EPM2A / Laforin, Polyclonal Antibody

 Also Known As   

Goat Polyclonal to Human EPM2A / Laforin

 Product Synonym Names    Anti-EPM2A / Laforin Antibody (Internal) IHC-plus; EPM2A; EPM2; LAFPTPase; LDE; Lafora PTPase; MELF; Laforin; LD; Human EPM2A; Laforin
 Product Gene Name   

anti-EPM2A antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 OMIM    phenotype 607566
 3D Structure    ModBase 3D Structure for O95278
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 Clonality    Polyclonal
 Host    Goat
 Species Reactivity    Bat, Gibbon, Bovine, Dog, Human, Monkey, Mouse, Pig, Rabbit, Rat
Predicted Reactivity: Xenopus (at least 90% immunogen sequence identity)
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 Specificity    Human EPM2A. This antibody is expected to recognize isoform a (NP_005661.1) only.
 Purity/Purification    Immunoaffinity Purified
 Form/Format    Tris-buffered saline, pH 7.3, 0.5% BSA, 0.02% sodium azide
 Concentration    0.5 mg/ml (lot specific)
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 Target Species    Human
 Immunogen Description    Synthetic peptide C-EATGHTNEMKHTTD from an internal region of human EPM2A (NP_005661.1). Percent identity by BLAST analysis: Human, Gibbon, Monkey, Marmoset, Mouse, Rat, Elephant, Panda, Bovine, Bat, Dog, Rabbit, Pig, Opossum (100%); Xenopus (93%).
 Immunogen Type    Synthetic peptide
 Immunogen    EPM2A / Laforin antibody was raised against synthetic peptide C-EATGHTNEMKHTTD from an internal region of human EPM2A (NP_005661.1). Percent identity by BLAST analysis: Human, Gibbon, Monkey, Marmoset, Mouse, Rat, Elephant, Panda, Bovine, Bat, Dog, Rabbit, Pig, Opossum (100%); Xenopus (93%).
 Antigen Modification    Internal
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 Preparation and Storage    Store at -20 degree C. Minimize freezing and thawing.
 Other Notes    Small volumes of anti-EPM2A antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Product Description specifically for anti-EPM2A antibody

   Lafora's disease, an autosomal recessive progressive myoclonus epilepsy, is caused by mutations in exons 1 and 4 of EPM2A gene. EPM2A encodes laforin which is a dual-specificity protein phosphatase located at the plasma membrane and the endoplasmic reticulum and is associated primarily with polyribosomes. EPM2A is functionally conserved in mammals and is involved in growth and maturation of neural networks.
 Product Categories/Family for anti-EPM2A antibody    Family: Protein Phosphatase
Subfamily: Laforin
 Applications Tested/Suitable for anti-EPM2A antibody   

Immunohistochemistry (IHC - Paraffin), Western Blot (WB), ELISA (EIA)

 Application Notes for anti-EPM2A antibody    ELISA (1:16000, 0.1 - 0.3 ug/ml), IHC-P (2.5 - 3.75 ug/ml), WB (0.1 - 0.3 ug/ml)
Usage: Peptide ELISA: antibody detection limit dilution 1:16000. Western Blot: Approx. 38kD band observed in Human Brain (Cerebellum), Mouse Brain and Rat Brain lysates (calculated MW of 37.2kD according to NP_005661.1). Recommended concentration: 0.1-0.3 ug/ml.
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 Immunohistochemistry (IHC) of anti-EPM2A antibody    Anti-EPM2A antibody IHC of human brain, cerebellum. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody concentration 2.5 ug/ml.
anti-EPM2A antibody Immunohistochemistry (IHC) (IHC) image
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 Immunohistochemistry (IHC) of anti-EPM2A antibody    Anti-EPM2A antibody IHC of human heart. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody concentration 2.5 ug/ml.
anti-EPM2A antibody Immunohistochemistry (IHC) (IHC) image
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 Western Blot (WB) of anti-EPM2A antibody    Antibody (0.1 ug/ml) staining of Human Cerebellum lysate (35 ug protein in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence.
anti-EPM2A antibody Western Blot (WB) (WB) image
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NCBI/Uniprot data below describe general gene information for EPM2A. It may not necessarily be applicable to this product.
 NCBI GI #    11321613
 NCBI GeneID    7957
 NCBI Accession #    NP_005661.1 [Other Products]
 NCBI GenBank Nucleotide #    NM_005670.3 [Other Products]
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 UniProt Primary Accession #    O95278 [Other Products]
 UniProt Secondary Accession #    O95483; Q5T3F5; Q6IS15; Q8IU96; Q8IX24; Q8IX25; Q9BS66; Q9UEN2; B3KMU5; B4DRZ2 [Other Products]
 UniProt Related Accession #    O95278; B3EWF7 [Other Products]
 Molecular Weight    35,169 Da
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 NCBI Official Full Name    laforin isoform a
 NCBI Official Synonym Full Names    epilepsy, progressive myoclonus type 2A, Lafora disease (laforin)
 NCBI Official Symbol    EPM2A [Similar Products]
 NCBI Official Synonym Symbols   
EPM2; MELF
[Similar Products]
 NCBI Protein Information    laforin; EPM2A; LAFPTPase; lafora PTPase; glucan phosphatase; epilepsy, progressive myoclonus type 2, Lafora disease (laforin)
 UniProt Protein Name    Laforin
 UniProt Synonym Protein Names   
Glucan phosphatase; Lafora PTPase; LAFPTPase
 Protein Family    Laforin
 UniProt Gene Name    EPM2A [Similar Products]
 UniProt Synonym Gene Names    LAFPTPase [Similar Products]
 UniProt Entry Name    EPM2A_HUMAN
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 NCBI Summary for EPM2A    This gene encodes a dual-specificity phosphatase that associates with polyribosomes. The encoded protein may be involved in the regulation of glycogen metabolism. Mutations in this gene have been associated with myoclonic epilepsy of Lafora. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jul 2008]
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 UniProt Comments for EPM2A    laforin: Dual specificity protein phosphatase. May be involved in the control of glycogen metabolism, particularly in monitoring for and preventing the formation of poorly branched glycogen molecules (polyglucosans). Acts as a scaffold protein to facilitate PPP1R3C/PTG ubiquitination by NHLRC1/malin. Forms a complex with NHLRC1/malin and HSP70 and this complex suppresses the cellular toxicity of misfolded proteins by promoting their degradation through the ubiquitin-proteasome system (UPS). Isoform 2, an inactive phosphatase, could function as a dominant-negative regulator for the phosphatase activity of isoform 1. Defects in EPM2A are a cause of progressive myoclonic epilepsy type 2 (EPM2); also known as Lafora disease. EPM2 is an autosomal recessive and severe form of adolescent-onset progressive epilepsy. Typically, as seizures increase in frequency, cognitive function declines towards dementia, and affected individuals die usually within 10 years after onset. EPM2 occurs worldwide, but it is particularly common in the mediterranean countries of southern Europe and northern Africa, in southern India and in the Middle East. At the cellular level, it is characterized by accumulation of starch-like polyglucosans called Lafora bodies (LBs) that are most abundant in organs with the highest glucose metabolism: brain, heart, liver and skeletal muscle. Among other conditions involving polyglucosans, EPM2 is unique in that the inclusions are in neuronal dendrites but not axons and the forming polyglucosan fibrils are associated with the endoplasmic reticulum. Belongs to the protein-tyrosine phosphatase family. 9 isoforms of the human protein are produced by alternative splicing.

Protein type: Motility/polarity/chemotaxis; Protein phosphatase, dual-specificity; EC 3.1.3.16; EC 3.1.3.48

Chromosomal Location of Human Ortholog: 6q24

Cellular Component: polysome; endoplasmic reticulum; cytoplasm; plasma membrane; cytosol; nucleus

Molecular Function: protein binding; phosphoinositide 5-phosphatase activity; protein tyrosine/serine/threonine phosphatase activity; protein tyrosine phosphatase activity; carbohydrate phosphatase activity; protein serine/threonine phosphatase activity

Biological Process: glycogen metabolic process; nervous system development; glycogen biosynthetic process; habituation; inositol phosphate dephosphorylation; carbohydrate metabolic process; glucose metabolic process; autophagy; pathogenesis; protein amino acid dephosphorylation

Disease: Myoclonic Epilepsy Of Lafora
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 Research Articles on EPM2A    1. Studied the role of conformational changes in human laforin structure due to existing single mutation W32G and prepared double mutation W32G/K87A related to loss of glycogen binding.
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with anti-EPM2A antibodyPathways associated with anti-EPM2A antibody
 Reference Product  PubMed Publications
 NHLRC1 antibody  >83 publications with EPM2A and NHLRC1
 EPM2AIP1 antibody  >8 publications with EPM2A and EPM2AIP1
 PPP1R3C antibody  >7 publications with EPM2A and PPP1R3C
 NFU1 antibody  >2 publications with EPM2A and NFU1
 PRKAB2 antibody  >1 publications with EPM2A and PRKAB2
 Products by Pathway  Pathway Diagram
 Disease Pathway antibodies  Disease Pathway Diagram
 Glucose Metabolism Pathway antibodies  Glucose Metabolism Pathway Diagram
 Glycogen Storage Diseases Pathway antibodies  Glycogen Storage Diseases Pathway Diagram
 Glycogen Synthesis Pathway antibodies  Glycogen Synthesis Pathway Diagram
 Metabolism Pathway antibodies  Metabolism Pathway Diagram
 Metabolism Of Carbohydrates Pathway antibodies  Metabolism Of Carbohydrates Pathway Diagram
 Myoclonic Epilepsy Of Lafora Pathway antibodies  Myoclonic Epilepsy Of Lafora Pathway Diagram
Diseases associated with anti-EPM2A antibodyOrgans/Tissues associated with anti-EPM2A antibody
 Disease Name  Pubmed Publications
 Lafora Disease Antibodies  >395 publications with EPM2A and Lafora Disease
 Brain Diseases Antibodies  >349 publications with EPM2A and Brain Diseases
 Neurodegenerative Diseases Antibodies  >224 publications with EPM2A and Neurodegenerative Diseases
 Seizures Antibodies  >86 publications with EPM2A and Seizures
 Disease Models, Animal Antibodies  >26 publications with EPM2A and Disease Models, Animal
 Liver Diseases Antibodies  >11 publications with EPM2A and Liver Diseases
 Neurobehavioral Manifestations Antibodies  >9 publications with EPM2A and Neurobehavioral Manifestations
 Congenital Abnormalities Antibodies  >8 publications with EPM2A and Congenital Abnormalities
 Movement Disorders Antibodies  >8 publications with EPM2A and Movement Disorders
 Nerve Degeneration Antibodies  >7 publications with EPM2A and Nerve Degeneration
 Organ/Tissue Name  Pubmed Publications
 Brain Antibodies  >158 publications with EPM2A and Brain
 Skin Antibodies  >77 publications with EPM2A and Skin
 Muscle Antibodies  >70 publications with EPM2A and Muscle
 Liver Antibodies  >47 publications with EPM2A and Liver
 Heart Antibodies  >26 publications with EPM2A and Heart
 Eye Antibodies  >7 publications with EPM2A and Eye
 Kidney Antibodies  >4 publications with EPM2A and Kidney
 Vascular Antibodies  >2 publications with EPM2A and Vascular
 Thyroid Antibodies  >1 publications with EPM2A and Thyroid
 Thymus Antibodies  >1 publications with EPM2A and Thymus
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