SUCLA2 Antibody

說明書

中文名稱：

SUCLA2兔多克隆抗體
貨號：

CSB-PA022917GA01HU
規格：

￥3,900
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

Uniprot No.：

Q9P2R7
基因名：

SUCLA2
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
宿主：

Rabbit
反應種屬：

Human,Mouse,Rat
免疫原：

Human SUCLA2
免疫原種屬：

Homo sapiens (Human)
抗體亞型：

IgG
純化方式：

Antigen Affinity Purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
產品提供形式：

Liquid
應用范圍：

ELISA,WB,IHC
Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Immunohistochemistry (IHC) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

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.
基因功能參考文獻：
1. SUCLA2 mutation is associated with Down syndrome and mitochondrial depletion syndrome. PMID: 28749033
2. The patient's cells lack the SUCLG1 protein, with significantly reduced levels of SUCLA2 and SUCLG2 protein PMID: 27484306
3. 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
4. SUCLA2 mutations should be analyzed in patients with slowly progressive encephalomyopathy, even in the absence of methylmalonic aciduria or mitochondrial DNA depletion. PMID: 24986829
5. This study demonstrated that SUCLA2 is expressed exclusively in neurons in the human cerebral cortex. PMID: 24085565
6. Identification of a novel mutation in SUCLA2 in two cousins affected with encephalomyopathy, is reported. PMID: 23759946
7. A novel homozygous mutation in SUCLA2 gene has been associated with severe mitochondrial encephalomyopathies in two Italian siblings. PMID: 23010432
8. 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
9. 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
10. Deficiency of SUCLA2 is associated with encephalomyopathy and mitochondrial DNA depletion. PMID: 15877282
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相關疾?。?/div>
Mitochondrial DNA depletion syndrome 5 (MTDPS5)
亞細胞定位：

Mitochondrion.
蛋白家族：

Succinate/malate CoA ligase beta subunit family, ATP-specific subunit beta subfamily
組織特異性：

Widely expressed. Not expressed in liver and lung.
數據庫鏈接：

HGNC: 11448

OMIM: 603921

KEGG: hsa:8803

STRING: 9606.ENSP00000367923

UniGene: Hs.743361

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