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PDSS2 Rabbit pAb (bs-10178R)  
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50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-10178R
英文名稱 PDSS2 Rabbit pAb
中文名稱 抑癌蛋白DLP1
別    名 ll-trans-decaprenyl-diphosphate synthase subunit 2; bA59I9.3; C6orf210; Candidate tumor suppressor protein; chromosome 6 open reading frame 210; Decaprenyl pyrophosphate synthase subunit 2; decaprenyl pyrophosphate synthetase subunit 2; Decaprenyl-diphosphate synthase subunit 2; DLP1; DLP1_HUMAN; hDLP1; Pdss2; prenyl(decaprenyl) diphosphate synthase, subunit 2; subunit 2 of decaprenyl diphosphate synthase.  
研究領(lǐng)域 腫瘤  免疫學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  細(xì)胞類型標(biāo)志物  線粒體  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
克 隆 號(hào)
交叉反應(yīng) (predicted: Rabbit,Pig,Dog,Horse)
產(chǎn)品應(yīng)用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ICC/IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 44 kDa
檢測(cè)分子量
細(xì)胞定位 細(xì)胞漿 線粒體
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from dog PDSS2: 31-130/399 
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 The protein encoded by this gene is an enzyme that synthesizes the prenyl side-chain of coenzyme Q, or ubiquinone, one of the key elements in the respiratory chain. The gene product catalyzes the formation of all trans-polyprenyl pyrophosphates from isopentyl diphosphate in the assembly of polyisoprenoid side chains, the first step in coenzyme Q biosynthesis. Defects in this gene are a cause of coenzyme Q10 deficiency.

Function:
Supplies decaprenyl diphosphate, the precursor for the side chain of the isoprenoid quinones ubiquinone-10.

Subunit:
Heterotetramer of 2 DPS1/TPRT and 2 DLP1 subunits.

Subcellular Location:
Mitochondrion (Potential).

DISEASE:
Defects in PDSS2 are the cause of coenzyme Q10 deficiency, primary, type 3 (COQ10D3) [MIM:614652]. A fatal encephalomyopathic form of coenzyme Q10 deficiency with nephritic syndrome. Coenzyme Q10 deficiency is an autosomal recessive disorder with variable manifestations consistent with 5 major phenotypes. The phenotypes include an encephalomyopathic form with seizures and ataxia; a multisystem infantile form with encephalopathy, cardiomyopathy and renal failure; a predominantly cerebellar form with ataxia and cerebellar atrophy; Leigh syndrome with growth retardation; and an isolated myopathic form.

Similarity:
Belongs to the FPP/GGPP synthase family.

Database links:

Entrez Gene: 57107 Human

Entrez Gene: 71365 Mouse

Entrez Gene: 365592 Rat

Omim: 610564 Human

SwissProt: Q86YH6 Human

SwissProt: Q33DR3 Mouse

SwissProt: Q5U2R1 Rat

Unigene: 486095 Human

Unigene: 363225 Mouse

Unigene: 20063 Rat



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