PRKAG2 Antibody
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中文名稱：PRKAG2兔多克隆抗體
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貨號：CSB-PA726942
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規格：￥1090
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其他：
產品詳情
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Uniprot No.：
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基因名：
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別名：PRKAG25'-AMP-activated protein kinase subunit gamma-2 antibody; AMPK gamma2 antibody; AMPK subunit gamma-2 antibody; H91620p antibody
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宿主：Rabbit
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反應種屬：Human,Mouse
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免疫原：Synthesized peptide derived from the N-terminal region of Human AMPKγ2.
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免疫原種屬：Homo sapiens (Human)
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標記方式：Non-conjugated
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抗體亞型：IgG
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純化方式：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
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產品提供形式：Liquid
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應用范圍：WB, IHC, IF, ELISA
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推薦稀釋比：
Application Recommended Dilution WB 1:500-1:2000 IHC 1:100-1:300 IF 1:200-1:1000 ELISA 1:20000 -
Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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貨期：Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
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靶點詳情
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功能：AMP/ATP-binding subunit of AMP-activated protein kinase (AMPK), an energy sensor protein kinase that plays a key role in regulating cellular energy metabolism. In response to reduction of intracellular ATP levels, AMPK activates energy-producing pathways and inhibits energy-consuming processes: inhibits protein, carbohydrate and lipid biosynthesis, as well as cell growth and proliferation. AMPK acts via direct phosphorylation of metabolic enzymes, and by longer-term effects via phosphorylation of transcription regulators. Also acts as a regulator of cellular polarity by remodeling the actin cytoskeleton; probably by indirectly activating myosin. Gamma non-catalytic subunit mediates binding to AMP, ADP and ATP, leading to activate or inhibit AMPK: AMP-binding results in allosteric activation of alpha catalytic subunit (PRKAA1 or PRKAA2) both by inducing phosphorylation and preventing dephosphorylation of catalytic subunits. ADP also stimulates phosphorylation, without stimulating already phosphorylated catalytic subunit. ATP promotes dephosphorylation of catalytic subunit, rendering the AMPK enzyme inactive.
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基因功能參考文獻：
- molecular screening for PRKAG2 mutations should be considered in patients who exhibit cardiac hypertrophy coexisting with ventricular pre-excitation. CMR offers promising advantages for evaluation of PRKAG2 cardiomyopathy. PMID: 28546535
- PRKAG2-mutated iPSC-CMs displayed functional and structural abnormalities, which were abolished by correcting the mutation in the patient's iPSCs using CRISPR technology. PMID: 28917552
- gamma2 AMPK activation downregulates fundamental sinoatrial cell pacemaker mechanisms to lower heart rate, including sarcolemmal hyperpolarization-activated current (I f) and ryanodine receptor-derived diastolic local subsarcolemmal Ca(2+) release. In contrast, loss of gamma2 AMPK induces a reciprocal phenotype of increased heart rate, and prevents the adaptive intrinsic bradycardia of endurance training. PMID: 29097735
- Case Report: PRKAG2 missense mutation causing glycogen storage disease and severe biventricular hypertrophy and high-grade atrio-ventricular block. PMID: 27496753
- We highlight the potential for patients with PRKAG2 mutations. PMID: 28801758
- This study of patients with PRKAG2 mutations provides a more comprehensive view of the natural history of this disease and demonstrates a high risk of cardiac complications. Early recognition of this disease appears important to allow an appropriate management. PMID: 28431061
- A novel missense genetic variant of unknown significance (GVUS) was detected in the PRKAG2 gene (c.869A>T, p.K290I). This novel GVUS has not been identified in any global population databases. PMID: 28690312
- As in patients with PRKAG2 cardiomyopathy, iPS cell and mouse models are protected from cardiac fibrosis, and we define a crosstalk between AMPK and post-transcriptional regulation of TGFbeta isoform signaling that has implications in fibrotic forms of cardiomyopathy. PMID: 28009297
- Identify a novel, de novo PRKAG2 mutation (K475E) in the cystathionine beta-synthase 3 repeat, a region critical for AMP binding, which affects AMP-activated protein kinase activity, activates cell growth pathways, and results in cardiac hypertrophy, which can be reversed with rapamycin. PMID: 28550180
- PRKAG2 polymorphism maybe important factor treating hypertensive patients with hydrochlorothiazide. PMID: 27381900
- Data suggest different gamma-isoforms in AMPK can have different effects on enzyme activation; here, activation of AMPK by compound 991 is greater if AMPK contains PRKAG2 versus PRKAG1 or PRKAG3. PMID: 28302767
- mice with chronic AMPK activation, resulting from mutation of the AMPK gamma2 subunit, exhibit ghrelin signaling-dependent hyperphagia, obesity, and impaired pancreatic islet insulin secretion. Humans bearing the homologous mutation manifest a congruent phenotype. PMID: 27133129
- PRKAG2 cardiac syndrome may present with eccentric distribution of LVH, involving focal mid-infero-lateral pattern in the early disease stage, and more diffuse pattern but focusing on interventricular septum in advanced cases. PMID: 26496977
- Overexpression of G100S mutation in PRKAG2 causes Wolff-Parkinson-White syndrome in transgenic zebrafish. PMID: 23992123
- Its mutation causes AMPK signaling abnormality which leads to cardiac syndrome. PMID: 23778007
- The PRKAG2 autosomal dominant cardiac syndrome may be commonly characterized by Left Ventricular Hypertrophy, an accessory pathway, and progression to conduction disease requiring implantation of a pacemaker. PMID: 23810891
- Data indicate that except AMPK-alpha1, expressions of the other five AMPK subunits -alpha2, -beta1, -beta2, -gamma1 and -gamma2 are significantly higher in ovarian carcinomas. PMID: 22897928
- Single nucleotide polymorphisms in PRKAG2 is associated with drug response in breast cancer. PMID: 23034890
- The authors found that the gene encoding the gamma2 subunit of AMP-activated protein kinase (AMPK) strongly correlated with Zaire Ebolavirus transduction in the tumor cell panel. PMID: 23115293
- found a significant association between the -26C/T polymorphism and cognitive impairment. Moreover, this polymorphism was also related to the presence of diabetes. PMID: 21813245
- The mutation in the PRKAG2 gene was identified as responsible for the familial form of WPW syndrome in this Chinese family. PMID: 20381067
- These two individuals may be considered to suffer from a combination of both a classical hypertrophic cardiomyopathy (due to the two mutations in MYBPC3) and a glycogen storage cardiomyopathy (due to the mutation in PRKAG2). PMID: 21409595
- no mutations were detected within the coding region of PRKAG2 in Wolff-Parkinson-White syndrome patients PMID: 20561859
- Newly identified polymorphisms (amino acid substitutions) are likely associated with cardiac disease in type 2 diabetic patients. PMID: 20022652
- This protein, transfected into a transgenic mouse, activates and mediates cardiac hypertrphic signaling pathways. PMID: 20005292
- PRKAG2 R302Q mutant induces AMPK activation and increases glycogen content in cardiomyocytes. PMID: 20031621
- Identified a novel mutation (Arg531Gly) in the PRKAG2 of AMP-activated protein kinase (AMPK) to be responsible for a syndrome associated with ventricular preexcitation and early onset of atrial fibrillation and conduction disease. PMID: 11748095
- The role of the causative gene, gamma-2 regulatory subunit (PRKAG2) of AMP-activated protein kinase, in the regulation of the glucose metabolic pathway in muscle suggests that genetic defects in PRKAG2 may induce a cardiac glycogenosis syndrome. PMID: 12015471
- Three of the mutations studied occur within the cystathionine beta-synthase (CBS) domains of gamma(2). Two of these mutations lead to a marked decrease in AMP dependence, whereas the third reduces AMP sensitivity. PMID: 12397075
- unlike familial WPW syndrome, constitutional mutation of PRKAG2 is not commonly associated with sporadic WPW syndrome PMID: 12716108
- mutations affecting PRKAG2 are likely to confer a specific alteration of AMPK function of particular importance in the myocardium PMID: 14519435
- Transgenic mice expressing human mutant(TG(R302Q)) PRKAG2 gene with the cardiac-specific promoter alpha-myosin heavy chain have ventricular pre-excitation, prolonged QRS, excess cardiac glycogen and a distinct AV accessory pathway. PMID: 15611370
- The glycogen-storage cardiomyopathy produced by LAMP2 or PRKAG2 mutations resembles hypertrophic cardiomyopathy but is distinguished by electrophysiological abnormalities PMID: 15673802
- The AMP kinase disease is uncommon in HCM and is characterized by progressive conduction disease and cardiac hypertrophy and includes extracardiac manifestations such as a skeletal myopathy. PMID: 15766830
- Biochemical characterization of the recombinant R531Q mutant protein showed >100-fold reduction of binding affinities for the regulatory nucleotides AMP and ATP but an enhanced basal activity and increased phosphorylation of the alpha -subunit. PMID: 15877279
- The PRKAG2 N488I mutation causes inappropriate AMPK activation, which leads to glycogen accumulation and heart conduction system disease when transfected into mice. PMID: 16275868
- The study describe a 38-year-old man with a new heterozygous PRKAG2 mutation (Ser548Pro) manifesting by hypertrophic cardiomyopathy, severe conduction system abnormalities, and skeletal muscle glycogenosis. PMID: 16487706
- AMP-activated protein kinase is regulated by a pseudosubstrate sequence on the gamma subunit. PMID: 17255938
- Altered AMPK gamma 2 subunit activity under normal energetic status remodels the cardiac metabolic network to cause a unique form of glycogen storage disease in transgenic mice. PMID: 17431505
- four members of the same family with a very similar ECG pattern characterized by conduction defects and mutations in PRKAG2 gene PMID: 17483151
- Human mutations which disrupt the nucleotide-binding affinity of the gamma2 subunit lead to loss of inhibition by ATP and inappropriate activate AMP-Kinase under resting conditions. PMID: 17990392
- Gene analysis identified a R302Q mutation of the gamma2 subunit producing AMP protein kinase, coded by the gene PRKAG2, and associated with a heart conduction defect. PMID: 18033003
- data provide insight into mechanisms of cardiac PRKAG2 disease and suggest that glycogen-storage cardiomyopathy can be modulated by lowering glycogen content in the heart PMID: 18158359
- REVIEW. Compelling evidence exists that Prkag2 mutations cause a "gain of function" in basal AMPK activity, leading to excessive cellular glucose uptake and pathological glycogen storage in the heart, resulting in a potentially fatal cardiac phenotype. PMID: 18195183
- Mutational analysis of PRKAG2, LAMP2, and NKX2-5 genes in a cohort of 125 patients with accessory atrioventricular connection. PMID: 19533775
- Preexcitation associated with the R302Q mutation in PRKAG2 is associated with Mahaim fibers. PMID: 19808419
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相關疾病：Wolff-Parkinson-White syndrome (WPWS); Cardiomyopathy, familial hypertrophic 6 (CMH6); Glycogen storage disease of heart lethal congenital (GSDH)
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蛋白家族：5'-AMP-activated protein kinase gamma subunit family
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組織特異性：Isoform B is ubiquitously expressed except in liver and thymus. The highest level is detected in heart with abundant expression in placenta and testis.
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數據庫鏈接：
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