SUCLA2 Antibody
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中文名稱：SUCLA2兔多克隆抗體
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貨號：CSB-PA022917GA01HU
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規(guī)格：￥3,900
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其他：
產(chǎn)品詳情
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Uniprot No.：
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基因名：SUCLA2
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別名：A BETA antibody; A SCS antibody; ATP specific succinyl CoA synthetase subunit beta antibody; ATP specific succinyl CoA synthetase, beta subunit antibody; ATP-specific succinyl-CoA synthetase subunit beta antibody; Mitochondrial succinyl CoA ligase [ADP forming] subunit beta antibody; MTDPS5 antibody; Renal carcinoma antigen NY-REN-39 antibody; Renal carcinoma antigen NYREN39 antibody; SCS betaA antibody; SCS-betaA antibody; SUCB1_HUMAN antibody; Succinate CoA ligase (ADP forming) antibody; Succinate CoA ligase [ADP forming] subunit beta, mitochondrial, succinyl CoA ligase [ADP forming] subunit beta, mitochondrial antibody; Succinate CoA ligase ADP forming beta subunit antibody; Succinate CoA ligase beta subunit antibody; Succinyl CoA ligase [ADP-forming] subunit beta, mitochondrial antibody; Succinyl CoA synthetase beta A chain antibody; Succinyl-CoA ligase [ADP-forming] subunit beta, mitochondrial antibody; Succinyl-CoA synthetase beta-A chain antibody; SUCLA2 antibody
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宿主：Rabbit
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反應(yīng)種屬：Human,Mouse,Rat
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免疫原：Human SUCLA2
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免疫原種屬：Homo sapiens (Human)
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抗體亞型：IgG
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純化方式：Antigen Affinity Purified
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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：PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
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產(chǎn)品提供形式：Liquid
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應(yīng)用范圍：ELISA,WB,IHC
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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(chǎn)品評價(jià)
相關(guān)產(chǎn)品
靶點(diǎn)詳情
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功能：ATP-specific succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of ATP and thus represents the only step of substrate-level phosphorylation in the TCA. The beta subunit provides nucleotide specificity of the enzyme and binds the substrate succinate, while the binding sites for coenzyme A and phosphate are found in the alpha subunit.
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基因功能參考文獻(xiàn)：
- SUCLA2 mutation is associated with Down syndrome and mitochondrial depletion syndrome. PMID: 28749033
- The patient's cells lack the SUCLG1 protein, with significantly reduced levels of SUCLA2 and SUCLG2 protein PMID: 27484306
- Sucla2 is related to the developmental stages of mouse spermatogenesis. Knockdown of Sucla2 decreases the viability of mouse spermatocytes by inducing apoptosis via decreased mitochondrial function of the cells. PMID: 27766610
- SUCLA2 mutations should be analyzed in patients with slowly progressive encephalomyopathy, even in the absence of methylmalonic aciduria or mitochondrial DNA depletion. PMID: 24986829
- This study demonstrated that SUCLA2 is expressed exclusively in neurons in the human cerebral cortex. PMID: 24085565
- Identification of a novel mutation in SUCLA2 in two cousins affected with encephalomyopathy, is reported. PMID: 23759946
- A novel homozygous mutation in SUCLA2 gene has been associated with severe mitochondrial encephalomyopathies in two Italian siblings. PMID: 23010432
- X-linked sideroblastic anemia due to carboxyl-terminal ALAS2 mutations that cause loss of binding to the beta-subunit of succinyl-CoA synthetase (SUCLA2). PMID: 22740690
- Mitochondrial DNA depletion, encephalomyopathic form, with methylmalonic aciduria is associated with mutations in SUCLA2, the gene encoding a beta subunit of succinate-CoA ligase. PMID: 19526370
- Deficiency of SUCLA2 is associated with encephalomyopathy and mitochondrial DNA depletion. PMID: 15877282
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相關(guān)疾病：Mitochondrial DNA depletion syndrome 5 (MTDPS5)
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亞細(xì)胞定位：Mitochondrion.
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蛋白家族：Succinate/malate CoA ligase beta subunit family, ATP-specific subunit beta subfamily
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組織特異性：Widely expressed. Not expressed in liver and lung.
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數(shù)據(jù)庫鏈接：
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