EPM2A monoclonal antibody (M02), clone 6C6
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More Files
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Specification
Product Description
Mouse monoclonal antibody raised against a partial recombinant EPM2A.
Immunogen
EPM2A (NP_001018051, 101 a.a. ~ 199 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Sequence
GNGPHHDRCCTYNENNLVDGVYCLPIGHWIEATGHTNEMKHTTDFYFNIAGHQAMHYSRILPNIWLGSCPRQVEHVTIKLKHELGITAVMNFQTEWDIV
Host
Mouse
Reactivity
Human, Rat
Interspecies Antigen Sequence
Mouse (95); Rat (97)
Isotype
IgG2a Kappa
Quality Control Testing
Antibody Reactive Against Recombinant Protein.
Western Blot detection against Immunogen (36.63 KDa) .
Storage Buffer
In 1x PBS, pH 7.4
Storage Instruction
Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
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Applications
Western Blot (Cell lysate)
EPM2A monoclonal antibody (M02), clone 6C6 Western Blot analysis of EPM2A expression in PC-12 ( Cat # L012V1 ).Western Blot (Transfected lysate)
Western Blot analysis of EPM2A expression in transfected 293T cell line by EPM2A monoclonal antibody (M02), clone 6C6.
Lane 1: EPM2A transfected lysate(37.2 KDa).
Lane 2: Non-transfected lysate.
Western Blot (Recombinant protein)
Sandwich ELISA (Recombinant protein)
Detection limit for recombinant GST tagged EPM2A is approximately 1ng/ml as a capture antibody.ELISA
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Gene Info — EPM2A
Entrez GeneID
7957GeneBank Accession#
NM_001018041Protein Accession#
NP_001018051Gene Name
EPM2A
Gene Alias
EPM2, MELF
Gene Description
epilepsy, progressive myoclonus type 2A, Lafora disease (laforin)
Gene Ontology
HyperlinkGene Summary
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
Other Designations
OTTHUMP00000017360|epilepsy, progressive myoclonus type 2, Lafora disease (laforin)|laforin
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Interactome
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Disease
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Publication Reference
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Lafora progressive myoclonus-epilepsy: Laforin targets malin to glycogen.
Sharmistha Mitra, Baozhi Chen, Peixiang Wang, Erin E Chown, Mathew Dear, Dikran R Guisso, Ummay Mariam, Jun Wu, Emrah Gumusgoz, Berge A Minassian.
Disease Models & Mechanisms 2023 Jan; 16(1):dmm049802.
Application:WB-Ti, Mouse , Mouse brain, Mouse heart, Mouse liver, Mouse muscle.
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Muscle glycogen remodeling and glycogen phosphate metabolism following exhaustive exercise of wild type and laforin knockout mice.
Irimia JM, Tagliabracci VS, Meyer CM, Segvich DM, DePaoli-Roach AA, Roach PJ.
The Journal of Biological Chemistry 2015 Jul; 290(37):22686.
Application:WB, Mouse, Skeletal muscle.
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The carbohydrate-binding domain of overexpressed STBD1 is important for its stability and protein-protein interactions.
Zhu Y, Zhang M, Kelly AR, Cheng A.
Bioscience Reports 2014 Jul; 34(4):e00117.
Application:WB-Ti, Mouse, Liver.
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A Bioassay for Lafora Disease and Laforin Glucan Phosphatase Activity.
Sherwood AR, Johnson MB, Delgado-Escueta AV, Gentry MS.
Clinical Biochemistry 2013 Dec; 46(18):1869.
Application:IP, Recombinant protein.
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Early-onset Lafora body disease.
Turnbull J, Girard JM, Lohi H, Chan EM, Wang P, Tiberia E, Omer S, Ahmed M, Bennett C, Chakrabarty A, Tyagi A, Liu Y, Pencea N, Zhao X, Scherer SW, Ackerley CA, Minassian BA.
Brain 2012 Sep; 135(Pt 9):2684.
Application:IEM, Mouse, Mouse skeletal muscle.
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Increased laforin and laforin binding to glycogen underlie Lafora body formation in malin-deficient Lafora disease.
Tiberia E, Turnbull J, Wang T, Ruggieri A, Zhao XC, Pencea N, Israelian J, Wang Y, Ackerley CA, Wang P, Liu Y, Minassian BA.
The Journal of Biological Chemistry 2012 Jul; 287(30):25650.
Application:IEM, WB, Mouse , Mouse brain.
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Laforin and malin knockout mice have normal glucose disposal and insulin sensitivity.
DePaoli-Roach AA, Segvich DM, Meyer CM, Rahimi Y, Worby CA, Gentry MS, Roach PJ.
Human Molecular Genetics 2012 Apr; 21(7):1604.
Application:WB-Ti, Mouse, Skeletal muscle.
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Glycogen hyperphosphorylation underlies lafora body formation.
Turnbull J, Wang P, Girard JM, Ruggieri A, Wang TJ, Draginov AG, Kameka AP, Pencea N, Zhao X, Ackerley CA, Minassian BA.
Annals of Neurology 2010 Dec; 68(6):925.
Application:WB-Ti, Mouse, Skeletal muscle, Brain.
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Laforin is a glycogen phosphatase, deficiency of which leads to elevated phosphorylation of glycogen in vivo.
Tagliabracci VS, Turnbull J, Wang W, Girard JM, Zhao X, Skurat AV, Delgado-Escueta AV, Minassian BA, Depaoli-Roach AA, Roach PJ.
PNAS 2007 Nov; 104(49):19262.
Application:WB, Mouse, Mouse brain.
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Lafora progressive myoclonus-epilepsy: Laforin targets malin to glycogen.
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