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Rabbit Anti-PDSS2/RBITC Conjugated antibody (bs-10178R-RBITC)
訂購(gòu)熱線:400-901-9800
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-10178R-RBITC
英文名稱 Rabbit Anti-PDSS2/RBITC Conjugated antibody
中文名稱 羅丹明(RBITC)標(biāo)記的抑癌蛋白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.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買        大包裝/詢價(jià)
說 明 書 100ul  
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
交叉反應(yīng) (predicted: Dog, Pig, Horse, Rabbit, )
產(chǎn)品應(yīng)用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 44kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from dog PDSS2
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產(chǎn)品介紹 background:
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



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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