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Rabbit Anti-SUCLA2/Renal carcinoma antigen NYREN39  antibody (bs-12838R)  
~~~促銷代碼KT202411~~~
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產(chǎn)品編號(hào) bs-12838R
英文名稱 Rabbit Anti-SUCLA2/Renal carcinoma antigen NYREN39  antibody
中文名稱 腎細(xì)胞癌抗原nyren39抗體
別    名 ATP specific succinyl CoA synthetase subunit beta; ATP-specific succinyl-CoA synthetase subunit beta; Renal carcinoma antigen NY-REN-39; Renal carcinoma antigen NYREN39; SCS betaA; SCS-betaA; SUCB1_HUMAN; 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.  
研究領(lǐng)域 細(xì)胞生物  神經(jīng)生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  干細(xì)胞  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human,Mouse (predicted: Rat,Rabbit,Pig,Cow,Chicken,Dog,Horse,Chimpanzee)
產(chǎn)品應(yīng)用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 45kDa
細(xì)胞定位 細(xì)胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SUCLA2: 221-320/463 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項(xiàng) This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 Succinyl-CoA synthetase (SCS) is a mitochondrial matrix enzyme that acts as a heterodimer, being composed of an invariant alpha subunit and a substrate-specific beta subunit. The protein encoded by this gene is an ATP-specific SCS beta subunit that dimerizes with the SCS alpha subunit to form SCS-A, an essential component of the tricarboxylic acid cycle. SCS-A hydrolyzes ATP to convert succinate to succinyl-CoA. Defects in this gene are a cause of myopathic mitochondrial DNA depletion syndrome. A pseudogene of this gene has been found on chromosome 6. [provided by RefSeq, Jul 2008]

Function:
Catalyzes the ATP-dependent ligation of succinate and CoA to form succinyl-CoA.

Subcellular Location:
Mitochondrion.

Tissue Specificity:
Widely expressed.

DISEASE:
Defects in SUCLA2 are the cause of mitochondrial DNA depletion syndrome type 5 (MTDPS5) [MIM:612073]. A disorder characterized by infantile onset of hypotonia, neurologic deterioration, a hyperkinetic-dystonic movement disorder, external ophthalmoplegia, deafness, variable renal tubular dysfunction, and mild methylmalonic aciduria.

Similarity:
Belongs to the succinate/malate CoA ligase beta subunit family.
Contains 1 ATP-grasp domain.

SWISS:
Q9P2R7

Gene ID:
8803

Database links:

Entrez Gene: 8803 Human

Entrez Gene: 20916 Mouse

Entrez Gene: 361071 Rat

Omim: 603921 Human

SwissProt: Q9P2R7 Human

SwissProt: Q9Z2I9 Mouse

SwissProt: B2RZ24 Rat

Unigene: 546323 Human

Unigene: 38951 Mouse



產(chǎn)品圖片
Sample: Hela(Human) Cell Lysate at 30 ug Raw264.7(Mouse) Cell Lysate at 30 ug Primary: Anti-SUCLA2 (bs-12838R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 45 kD Observed band size: 53 kD
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