抑癌蛋白DLP1抗体
规格：1mg/1ml
英文名： PDSS2
别名： 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; decapreny
分子量： 44kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human PDSS2
交叉反应：Human, Mouse, Rat,
抑癌蛋白DLP1抗体细胞定位：细胞浆 线粒体
产品介绍：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 抑癌蛋白DLP1抗体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. Gene ID: 57107 Database links: Entrez Gene: 57107 Human Entrez Gene: 抑癌蛋白DLP1抗体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.
抑癌蛋白DLP1抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 **学 信号转导 细胞类型标志物 线粒体
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid