Recombinant Human Succinate--CoA ligase [ADP-forming] subunit beta, mitochondrial (SUCLA2)

Code CSB-YP868398HU
MSDS
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Source Yeast
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Code CSB-EP868398HU-B
MSDS
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Source E.coli
Conjugate 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.
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Code CSB-BP868398HU
MSDS
Size Pls inquire
Source Baculovirus
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Code CSB-MP868398HU
MSDS
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Source Mammalian cell
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Product Details

Purity
>85% (SDS-PAGE)
Target Names
SUCLA2
Uniprot No.
Alternative Names
A BETA; A SCS; ATP specific succinyl CoA synthetase subunit beta; ATP specific succinyl CoA synthetase, beta subunit; ATP-specific succinyl-CoA synthetase subunit beta; Mitochondrial succinyl CoA ligase [ADP forming] subunit beta; MTDPS5; Renal carcinoma antigen NY-REN-39; Renal carcinoma antigen NYREN39; SCS betaA; SCS-betaA; SUCB1_HUMAN; Succinate CoA ligase (ADP forming); Succinate CoA ligase [ADP forming] subunit beta, mitochondrial, succinyl CoA ligase [ADP forming] subunit beta, mitochondrial; Succinate CoA ligase ADP forming beta subunit; Succinate CoA ligase beta subunit; Succinyl CoA ligase [ADP-forming] subunit beta, mitochondrial; Succinyl CoA synthetase beta A chain; Succinyl-CoA ligase [ADP-forming] subunit beta, mitochondrial; Succinyl-CoA synthetase beta-A chain; SUCLA2
Species
Homo sapiens (Human)
Expression Region
53-463
Target Protein Sequence
LSLHEYMS MELLQEAGVS VPKGYVAKSP DEAYAIAKKL GSKDVVIKAQ VLAGGRGKGT FESGLKGGVK IVFSPEEAKA VSSQMIGKKL FTKQTGEKGR ICNQVLVCER KYPRREYYFA ITMERSFQGP VLIGSSHGGV NIEDVAAESP EAIIKEPIDI EEGIKKEQAL QLAQKMGFPP NIVESAAENM VKLYSLFLKY DATMIEINPM VEDSDGAVLC MDAKINFDSN SAYRQKKIFD LQDWTQEDER DKDAAKANLN YIGLDGNIGC LVNGAGLAMA TMDIIKLHGG TPANFLDVGG GATVHQVTEA FKLITSDKKV LAILVNIFGG IMRCDVIAQG IVMAVKDLEI KIPVVVRLQG TRVDDAKALI ADSGLKILAC DDLDEAARMV VKLSEIVTLA KQAHVDVKFQ LPI
Protein Length
Full Length of Mature Protein
Tag Info
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.
Form
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.
Buffer before Lyophilization
Tris/PBS-based buffer, 6% Trehalose, pH 8.0
Reconstitution
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.
Troubleshooting and FAQs
Storage Condition
Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
Shelf Life
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.
Lead Time
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.
Notes
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.

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Target Background

Function
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.
Gene References into Functions
  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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Involvement in disease
Mitochondrial DNA depletion syndrome 5 (MTDPS5)
Subcellular Location
Mitochondrion.
Protein Families
Succinate/malate CoA ligase beta subunit family, ATP-specific subunit beta subfamily
Tissue Specificity
Widely expressed. Not expressed in liver and lung.
Database Links

HGNC: 11448

OMIM: 603921

KEGG: hsa:8803

STRING: 9606.ENSP00000367923

UniGene: Hs.743361

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