Key features and details
- Goat polyclonal to EPM2A/Laforin
- Suitable for: IHC-P, WB
- Reacts with: Mouse, Rat, Human
- Isotype: IgG
参阅全部 EPM2A/Laforin 一抗
特异性ab129321 is expected to recognize isoform 1 (NP_005661.1) only.
经测试应用适用于: IHC-P, WBmore details
种属反应性与反应: Mouse, Rat, Human
预测可用于: Rabbit, Cow, Dog, Pig, Xenopus laevis, a wide range of other species
- Human brain, cerebellum and heart tissues; Human Cerebellum lysate.
This product was previously labelled as EPM2A', 'EPM2A, isoform 9
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存放说明Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
Preservative: 0.02% Sodium azide
Constituents: 99% Tris buffered saline, 0.5% BSA
Concentration information loading...
纯度Immunogen affinity purified
Our Abpromise guarantee covers the use of ab129321 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|IHC-P||Use a concentration of 2.5 - 3.75 µg/ml. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.|
|WB||Use a concentration of 0.1 - 0.3 µg/ml. Predicted molecular weight: 37 kDa.|
功能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.
组织特异性Expressed in heart, skeletal muscle, kidney, pancreas and brain. Isoform 4 is also expressed in the placenta.
疾病相关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.
序列相似性Belongs to the protein-tyrosine phosphatase family.
Contains 1 CBM20 (carbohydrate binding type-20) domain.
Contains 1 tyrosine-protein phosphatase domain.
翻译后修饰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.
细胞定位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
ab129321 at 3.75 µg/ml staining EPM2A/Laforin in Formalin-Fixed, Paraffin-Embedded Human brain, cerebellum tissue by immunohistochemistry.
ab129321 at 3.75 µg/ml staining EPM2A/Laforin in Formalin-Fixed, Paraffin-Embedded Human heart tissue by immunohistochemistry.
Anti-EPM2A/Laforin antibody (ab129321) at 0.1 µg/ml + Human Cerebellum lysate in RIPA buffer at 35 µg
Developed using the ECL technique.
Predicted band size: 37 kDa