Product Name	Anti-PDSS2/AF594
Chinese Name	AF594标记的抑癌蛋白DLP1
Alias	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.
Research Area	Tumour  immunology  Signal transduction  Cell type markers  Mitochondrion
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	(predicted: Dog, Pig, Horse, Rabbit, )
Applications	ICC=1:50-200 IF=1:50-200  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Molecular weight	44kDa
Form	Lyophilized or Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from dog PDSS2
Lsotype	IgG
Purification	affinity purified by Protein A
Storage Buffer	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	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.
Product Detail	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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