Recombinant Human Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial (SUCLG1)

中文名稱：

人SUCLG1重組蛋白
貨號：

CSB-YP022919HU
說明書：

MSDS Datasheet
規格：
來源：

Yeast
其他：

中文名稱：

人SUCLG1重組蛋白
貨號：

CSB-EP022919HU
說明書：

MSDS Datasheet
規格：
來源：

E.coli
其他：

中文名稱：

人SUCLG1重組蛋白
貨號：

CSB-EP022919HU-B
說明書：

MSDS Datasheet
規格：
來源：

E.coli
共軛：

Avi-tag Biotinylated
E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.
其他：

中文名稱：

人SUCLG1重組蛋白
貨號：

CSB-BP022919HU
說明書：

MSDS Datasheet
規格：
來源：

Baculovirus
其他：

中文名稱：

人SUCLG1重組蛋白
貨號：

CSB-MP022919HU
說明書：

MSDS Datasheet
規格：
來源：

Mammalian cell
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

純度：

>85% (SDS-PAGE)
基因名：

SUCLG1
Uniprot No.：

P53597
別名：

FLJ21114; FLJ43513; GALPHA; MTDPS9; SCS alpha; SCS-alpha; SUCA_HUMAN; Succinate CoA ligase alpha subunit ; Succinyl CoA ligase [GDP forming] subunit alpha mitochondrial; Succinyl CoA synthetase subunit alpha; Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha; mitochondrial; Succinyl-CoA synthetase subunit alpha; SUCLA1; SUCLG 1; SUCLG1
種屬：

Homo sapiens (Human)
蛋白長度：

Full length protein
表達區域：

41-346
氨基酸序列

CSYTASRQHL YVDKNTKIIC QGFTGKQGTF HSQQALEYGT KLVGGTTPGK GGQTHLGLPV FNTVKEAKEQ TGATASVIYV PPPFAAAAIN EAIEAEIPLV VCITEGIPQQ DMVRVKHKLL RQEKTRLIGP NCPGVINPGE CKIGIMPGHI HKKGRIGIVS RSGTLTYEAV HQTTQVGLGQ SLCVGIGGDP FNGTDFIDCL EIFLNDSATE GIILIGEIGG NAEENAAEFL KQHNSGPNSK PVVSFIAGLT APPGRRMGHA GAIIAGGKGG AKEKISALQS AGVVVSMSPA QLGTTIYKEF EKRKML
蛋白標簽：

Tag?type?will?be?determined?during?the?manufacturing?process.

The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
產品提供形式：

Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
復溶：

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
儲存條件：

Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
保質期：

The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
貨期：

Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
注意事項：

Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet ：

Please contact us to get it.

產品評價

靶點詳情

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