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Product Name PDSS2 Chinese Name 抑癌蛋白DLP1抗体 Alias 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. Research Area Tumour immunology Signal transduction Cell type markers Mitochondrion Immunogen Species Rabbit Clonality Polyclonal React Species Human, (predicted: Mouse, Rat, ) Applications ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 44kDa Cellular localization cytoplasmic Mitochondrion Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human PDSS2: 21-100/399 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail 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
Product Picture Paraformaldehyde-fixed, paraffin embedded (human Pancreatic cancer); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (PDSS2) Polyclonal Antibody, Unconjugated (SL0376R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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