製品名Anti-EPM2A/Laforin antibody [EP7235] - BSA and Azide free
EPM2A/Laforin 一次抗体 製品一覧
製品の詳細Rabbit monoclonal [EP7235] to EPM2A/Laforin - BSA and Azide free
アプリケーション適用あり: ICC/IF, IHC-P, Flow Cyt, WBmore details
Synthetic peptide within Human EPM2A/Laforin aa 1-100 (N terminal). The exact sequence is proprietary.
ab248308 is the carrier-free version of ab129110 This format is designed for use in antibody labeling, including fluorochromes, metal isotopes, oligonucleotides, enzymes.
Our carrier-free formats are supplied in a buffer free of BSA, sodium azide and glycerol for higher conjugation efficiency.
Use our conjugation kits for antibody conjugates that are ready-to-use in as little as 20 minutes with <1 minute hands-on-time and 100% antibody recovery: available for fluorescent dyes, HRP, biotin and gold.
Ab248308 is compatible with the Maxpar® Antibody Labeling Kit from Fluidigm.
Maxpar® is a trademark of Fluidigm Canada Inc.
Mouse, Rat: We have preliminary internal testing data to indicate this antibody may not react with these species. Please contact us for more information. This product was previously labelled as EPM2A', 'EPM2A, isoform 9
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents.
This product is a recombinant rabbit monoclonal antibody.
保存方法Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Concentration information loading...
Our Abpromise guarantee covers the use of ab248308 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|ICC/IF||Use at an assay dependent concentration.|
|IHC-P||Use at an assay dependent concentration. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.|
|Flow Cyt||Use at an assay dependent concentration.|
|WB||Use at an assay dependent concentration. Detects a band of approximately 35 kDa (predicted molecular weight: 37 kDa).|
機能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.
組織特異性Expressed in heart, skeletal muscle, kidney, pancreas and brain. Isoform 4 is also expressed in the placenta.
関連疾患Defects in EPM2A are a cause of progressive myoclonic epilepsy type 2 (EPM2) [MIM:254780]; 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.
配列類似性Belongs to the protein-tyrosine phosphatase family.
Contains 1 CBM20 (carbohydrate binding type-20) domain.
Contains 1 tyrosine-protein phosphatase domain.
翻訳後修飾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.
細胞内局在Cytoplasm; Cytoplasm. Nucleus; Endoplasmic reticulum. Cell membrane. Nucleus. Also found in the nucleus; Endoplasmic reticulum. Cell membrane. Primarily associated with polyribosomes at the endoplasmic reticulum, also found at the plasma membrane and Cytoplasm. Under glycogenolytic conditions localizes to the nucleus.
- Information by UniProt
- Epilepsy progressive myoclonus type 2 Lafora disease (laforin) antibody
- Epilepsy progressive myoclonus type 2A Lafora disease (laforin) antibody
- EPM2 antibody
ab248308 has not yet been referenced specifically in any publications.