SUCLG1 Antibody, FITC conjugated

說明書

中文名稱：

SUCLG1兔多克隆抗體, FITC偶聯
貨號：

CSB-PA022919LC01HU
規格：

￥880
其他：

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靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) SUCLG1 Polyclonal antibody
Uniprot No.：

P53597
基因名：

SUCLG1
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
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Succinate--CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial protein (210-316AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

FITC
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

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

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
產品提供形式：

Liquid
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.

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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.
基因功能參考文獻：
1. 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
2. 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
3. Long survival, to age 20 years or older, was reported in 12% of SUCLA2 and in 10% of SUCLG1 patients. PMID: 26475597
4. First Chinese report of succinyl-CoA ligase deficiency caused by novel SUCLG1 mutations; five novel pathogenic mutations in SUCLG1 were identified PMID: 26028457
5. 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
6. 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
7. 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
8. 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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相關疾病：

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：

HGNC: 11449

OMIM: 245400

KEGG: hsa:8802

STRING: 9606.ENSP00000377446

UniGene: Hs.270428

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