產(chǎn)品編號(hào) | bs-0376R |
英文名稱 | PDSS2 Rabbit pAb |
中文名稱 | 抑癌蛋白DLP1抗體 |
別 名 | All-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 |
抗體來源 | Rabbit |
克隆類型 | Polyclonal |
交叉反應(yīng) | Human (predicted: Mouse,Rat) |
產(chǎn)品應(yīng)用 | IHC-P=1:100-500,IHC-F=1:100-500
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 human PDSS2: 21-100/399 |
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. SWISS: Q86YH6 Gene ID: 57107 Database links: Entrez Gene: 57107 Human Entrez Gene: 71365 Mouse Omim: 610564 Human SwissProt: Q86YH6 Human SwissProt: Q33DR3 Mouse Unigene: 486095 Human Unigene: 363225 Mouse Unigene: 20063 Rat |
產(chǎn)品圖片 | |
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