PLN Antibody
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货号:CSB-PA030091
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规格:¥880
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其他:
产品详情
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Uniprot No.:P26678
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基因名:
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别名:Cardiac phospholamban antibody; CMD1P antibody; CMH18 antibody; PLB antibody; Pln antibody; PPLA_HUMAN antibody
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宿主:Rabbit
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反应种属:Human,Mouse,Rat
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免疫原:Synthesized peptide derived from Human PLB around the non-phosphorylation site of S16/T17.
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免疫原种属:Homo sapiens (Human)
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标记方式:Non-conjugated
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抗体亚型:IgG
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纯化方式:The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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浓度:It differs from different batches. Please contact us to confirm it.
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保存缓冲液:Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
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产品提供形式:Liquid
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应用范围:IF, ELISA
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推荐稀释比:
Application Recommended Dilution IF 1:200-1:1000 ELISA 1:10000 -
Protocols:
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储存条件:Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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货期: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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靶点详情
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功能:Reversibly inhibits the activity of ATP2A2 in cardiac sarcoplasmic reticulum by decreasing the apparent affinity of the ATPase for Ca(2+). Modulates the contractility of the heart muscle in response to physiological stimuli via its effects on ATP2A2. Modulates calcium re-uptake during muscle relaxation and plays an important role in calcium homeostasis in the heart muscle. The degree of ATP2A2 inhibition depends on the oligomeric state of PLN. ATP2A2 inhibition is alleviated by PLN phosphorylation.
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基因功能参考文献:
- 2 lethal PLN mutations, R9C and R25C, which lead to dilated cardiomyopathy, were studied by biomolecular NMR. R25C enhances phospholmaban dynamics and shifts the conformational equilibrium toward the R state. R9C drives the amphipathic cytoplasmic domain toward the membrane-associate state, enriching the T state. PMID: 29501609
- Structure-Function Relationship of the SERCA Pump and Its Regulation by Phospholamban and Sarcolipin. PMID: 29594859
- The co-transfection of VHL and PLN in HEK293 cells decreased PLN expression under oxidative stress, whereas knockdown of VHL increased PLN expression both under normal and oxidative stress conditions. PMID: 29068413
- Hearts from patients with a p. Arg14del PLN mutation have a pattern of Right Ventricle Fibrofatty Replacement and Left Ventricular Fibrosis with fatty changes mostly in the posterolateral wall, independently of clinical presentation. PMID: 28365402
- LMOD1, SYNPO2, PDLIM7, PLN, and SYNM down-regulation reflect the altered phenotype of smooth muscle cells in vascular disease and could be early sensitive markers of SMC dedifferentiation. PMID: 27470516
- microRNAs (miRNAs) 1 and 21 bind PLN strongly and relieve PLN inhibition of SERCA to a greater extent than a similar length random sequence RNA mixture. PMID: 27531746
- Data suggest phospholamban (PLN) gene is a rare cause of cardiomyopathy in African patients. PMID: 26917049
- Phospholamban and sarcolipin are membrane proteins that differentially regulate SERCA function. (Review) PMID: 26743715
- PLN may be a key molecular player in rigid substrate-induced cellular hypertrophy in eosinophilic esophagitis. PMID: 26542032
- These data suggest that PLN is, at least partially, oligo-ubiquitinated at Lys(3) and degraded through Ser(16)-phosphorylation-mediated poly-ubiquitination during heart failure. PMID: 26966065
- hereditary mutants of phospholamban are associated with heart failure [review] PMID: 25563649
- PLN pentamers reduce phosphorylation of monomers at baseline and delay monomer phosphorylation upon PKA stimulation leading to increased interaction of PLN monomers with SERCA2a. PMID: 25562800
- Phospholamban R14del mutation carriers are at high risk for malignant ventricular arrhythmias and end-stage heart failure, with left ventricular ejection fraction <45% and sustained or nonsustained ventricular tachycardia as independent risk factors. PMID: 24909667
- Although SLN and PLB binding to SERCA have different functional outcomes on the coupling efficiency of SERCA, both proteins decrease the apparent Ca(2+) affinity of the pump, suggesting that SLN and PLB inhibit SERCA by using a similar mechanism. PMID: 25983321
- Phospholamban, and its interacting partners, regulates excitation contraction coupling and myocardial contraction. [Review] PMID: 25451386
- PLN mutations rarely cause cardiomyopathy PMID: 25928149
- analysis of how the conformational dynamics of protein kinase A induced by a lethal mutant of phospholamban hinder phosphorylation PMID: 25775607
- Aim of the present study is to determine the exact pattern of fibrosis and fatty replacement in PLN p.Arg14del mutation positive patients. PMID: 24732829
- Engineered upregulation of PLB expression in hESC/iPSC-vCMs restores a positive inotropic response to beta-adrenergic stimulation. PMID: 25504561
- a previously unrecognized mechanism for ESM cell contraction that depends on TGF-beta1, its receptors, and PLN. PMID: 24835503
- We conclude that PLB C-terminal residues are critical for localization, oligomerization, and regulatory function. In particular, the PLB C terminus is an important determinant of the quaternary structure of the SERCA regulatory complex. PMID: 25074938
- SLN and PLN are co-expressed in most fibers, which suggests that super-inhibition of SERCAs may be physiologically important in the regulation of intracellular Ca2+ in human skeletal muscle. PMID: 24358354
- Report PLN mutations in dilated cardiomyopathy. PMID: 24037902
- A PLN founder mutation and LMNA mutations were most prevalent and often demonstrated a specific phenotype in dilated cardiomyopathy patients PMID: 23349452
- PLN mutation carriers have ARVD/C characteristics, including important right ventricular involvement, and additionally more often low-voltage electrocardiograms, inverted T waves in the left precordial leads, and left ventricular involvement. PMID: 23871674
- In the context of data on PLN/SERCA interaction and on Ca(2+) accumulation in the sarcoplasmic reticulum the present results are consistent with the view that PLN channel activity could participate in the balancing of charge during Ca(2+) uptake. PMID: 23308118
- The researchers found evidence of an association between the phospholamban R14del and the presence of dilated or arrhythmogenic cardiomyopathies in a group of patients. PMID: 22820313
- 1,014 patients with heart failure screened for mutations in PLN gene; identified 4 unrelated patients with PLN mutations, 3 in same amino acid residue (R9); conclude mutations in PLN gene are rare cause of heart failure, present almost exclusively in patients with dilated cardiomyopathy etiology; Arg9 and Leu39 residues are leading location of mutations described to date PMID: 22137083
- human PLN-R14Del is misrouted to the sarcolemma, in the absence of endogenous PLN, and alters NKA activity, leading to cardiac remodeling. PMID: 22155237
- Hydrophobic imbalance in the cytoplasmic domain of phospholamban is a determinant for lethal dilated cardiomyopathy. PMID: 22427649
- TOAC spin labels placed on the WT-PLB transmembrane domain showed highly restricted motion with more than 100ns rotational correlation time (tau(c)); whereas the loop, and the cytoplasmic regions each consists of two distinct motional dynamics PMID: 22172806
- Characterizing phospholamban to sarco(endo)plasmic reticulum Ca2+-ATPase 2a (SERCA2a) protein binding interactions in human cardiac sarcoplasmic reticulum vesicles using chemical cross-linking. PMID: 22247554
- PLN generates canonical ion channel fluctuations with two conductance levels and a moderate cation selectivity PMID: 21687864
- both topology and function of PLN are shaped by the interactions with lipids, which fine-tune the regulation of SERCA PMID: 21576492
- PLN gene mutations were not found to be associated with HCM in the study group. PMID: 21332051
- Lethal Arg9Cys phospholamban mutation hinders Ca2+-ATPase regulation and phosphorylation by protein kinase A. PMID: 21282613
- Mutations in PLN are rare in frequency, yet the small size of the genetic locus may make it amenable to inclusion on HCM gene test panels. PMID: 21167350
- In this study, they investigated the effects of PLB phosphorylation and mutation on the interaction between a PLB oligomer and SERCA in the context of 2D crystals. PMID: 21108950
- Study conclude that PLN is enriched in the ER due to COP I-mediated transport that is dependent on its intact di-arginine motif and that the N-terminal di-arginine motif may act as a general ER retrieval sequence. PMID: 20634894
- sarcolipin binds to phospholamban and inhibits polymerization PMID: 12032137
- phosphorylation of phospholamban does not affect its structure and gives it more loose helical packing than if not phosphorylated PMID: 12080135
- Modeling of the inhibitory interaction of phospholamban with the Ca2+ ATPase. PMID: 12525698
- report that an inherited human dilated cardiomyopathy with refractory congestive heart failure is caused by a dominant Arg --> Cys missense mutation at residue 9 (R9C) in phospholamban PMID: 12610310
- role in regulating sarco(endo)plasmic reticulum Ca2+-ATPase by binding to transmembrane helices in conjunction with sarcolipin PMID: 12692302
- Mutation of the phospholamban promoter associated with hypertrophic cardiomyopathy. PMID: 12705874
- SERCA2a and phospholamban bind to S100A1 in the human heart PMID: 12804600
- The frequency-dependent phosphorylation of Ser16-PLB may favor an increase in Ca2+ transient and force generation in humans. PMID: 14530977
- This study concludes that phospholamban (PLB) increases the maximal activity (Vmax) of calcium (Ca2+)-ATPase, and that the magnitude of this effect is sensitive to mutation. A region of mutant PLB responsible for this regulatory property is identified. PMID: 15736939
- The unusual bellflower-like assembly is held together by leucine/isoleucine zipper motifs along the membrane-spanning helices. PMID: 16043693
- the nonreversible superinhibitory function of mutant PLN-R14Del may lead to inherited dilated cardiomyopathy and premature death in both humans and mice PMID: 16432188
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相关疾病:Cardiomyopathy, dilated 1P (CMD1P); Cardiomyopathy, familial hypertrophic 18 (CMH18)
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亚细胞定位:Endoplasmic reticulum membrane; Single-pass membrane protein. Sarcoplasmic reticulum membrane; Single-pass membrane protein. Mitochondrion membrane; Single-pass membrane protein. Membrane; Single-pass membrane protein.
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蛋白家族:Phospholamban family
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组织特异性:Heart muscle (at protein level).
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数据库链接:
HGNC: 9080
OMIM: 172405
KEGG: hsa:5350
STRING: 9606.ENSP00000350132
UniGene: Hs.170839
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