SUCLG1 Antibody
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中文名稱：SUCLG1兔多克隆抗體
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貨號：CSB-PA022919GA01HU
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規格：￥3,900
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其他：
產品詳情
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Uniprot No.：
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基因名：SUCLG1
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別名：FLJ21114 antibody; FLJ43513 antibody; GALPHA antibody; MTDPS9 antibody; SCS alpha antibody; SCS-alpha antibody; SUCA_HUMAN antibody; Succinate CoA ligase alpha subunit antibody; Succinyl CoA ligase [GDP forming] subunit alpha mitochondrial antibody; Succinyl CoA synthetase subunit alpha antibody; Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha; mitochondrial antibody; Succinyl-CoA synthetase subunit alpha antibody; SUCLA1 antibody; SUCLG 1 antibody; SUCLG1 antibody
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宿主：Rabbit
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反應種屬：Human,Mouse,Rat
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免疫原：Human SUCLG1
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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 0.02% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
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產品提供形式：Liquid
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應用范圍：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.
產品評價
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靶點詳情
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功能：Succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of either ATP or GTP and thus represents the only step of substrate-level phosphorylation in the TCA. The alpha subunit of the enzyme binds the substrates coenzyme A and phosphate, while succinate binding and specificity for either ATP or GTP is provided by different beta subunits.
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基因功能參考文獻：
- We report two Tunisian patients belonging to a consanguineous family with mitochondrial encephalomyopathy. Mutational analysis of SUCLG1 gene showed the presence of c.41T > C in exon 1 in a homozygous state. This mutation substitutes a conserved methionine residue to a threonine at position 14 (p.M14T) located at the SUCLG1 protein mitochondrial targeting sequence. PMID: 29217198
- These abnormal phenotypes are rescued upon ectopic expression of wild-type SUCLG1 in the patient's fibroblasts, thus functionally confirming the pathogenic nature of the SUCLG1 VUS identified in this patient and expanding the phenotypic spectrum for SUCLG1 deficiency PMID: 27484306
- Long survival, to age 20 years or older, was reported in 12% of SUCLA2 and in 10% of SUCLG1 patients. PMID: 26475597
- First Chinese report of succinyl-CoA ligase deficiency caused by novel SUCLG1 mutations; five novel pathogenic mutations in SUCLG1 were identified PMID: 26028457
- 3 novel mutations have been identified in patients with the mitochondrial DNA depletion syndrome (c.1048G>A and c.1049G>T in SUCLA2 and c.531+4A>T in SUCLG1). PMID: 22980518
- Our results suggest that SUCLG1 mutations that lead to complete absence of SUCLG1 protein are responsible for a very severe disorder with antenatal manifestations, whereas a SUCLA2-like phenotype is found in patients with residual SUCLG1 protein. PMID: 20693550
- This report enlarges the phenotypic spectrum of SUCLG1 mutations and confirms that a characteristic metabolic profile (presence of MMA and C4-DC carnitine in urines) and basal ganglia MRI lesions are the hallmarks of SCS defects. PMID: 20197121
- A novel mutation in the SUCLG1 gene was found in two newborns having lethal lactic acidosis, multi-organ failure and congenital malformations including interrupted aortic arch. PMID: 20227526
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相關疾?。?/div>Mitochondrial DNA depletion syndrome 9 (MTDPS9)亞細胞定位：Mitochondrion.蛋白家族：Succinate/malate CoA ligase alpha subunit family數據庫鏈接：
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