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Rabbit Anti-PDSS2/Gold Conjugated antibody (bs-10178R-Gold)
訂購熱線:400-901-9800
訂購郵箱:sales@xucheq.com
訂購QQ:  400-901-9800
技術(shù)支持:techsupport@xucheq.com
說 明 書: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-10178R-Gold
英文名稱1 Rabbit Anti-PDSS2/Gold Conjugated antibody
中文名稱 膠體金標記的抑癌蛋白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)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul(10nm  15nm  35nm
研究領(lǐng)域 腫瘤  免疫學  信號轉(zhuǎn)導(dǎo)  細胞類型標志物  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Dog, Pig, Horse, Rabbit, )
產(chǎn)品應(yīng)用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
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 0.4mg/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 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存條件 Store at 2-8 oC for 3-6 months. Avoid repeated freeze/thaw cycles.
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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