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PDE6C Antibody

  • 货号：
    CSB-PA23139A0Rb
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Western Blot
      Positive WB detected in: Hela whole cell lysate, SH-SY5Y whole cell lysate
      All lanes: PDE6C antibody at 3.7μg/ml
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 100 kDa
      Observed band size: 100 kDa
    • Immunofluorescence staining of A549 cells with CSB-PA23139A0Rb at 1:66, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) PDE6C Polyclonal antibody
  • Uniprot No.：
    P51160
  • 基因名：
    PDE6C
  • 别名：
    5''-cyclic phosphodiesterase subunit alpha'' antibody; cGMP phosphodiesterase 6C antibody; COD4 antibody; Cone cGMP specific 3' 5' cyclic phosphodiesterase subunit alpha' antibody; Cone cGMP-specific 3'' antibody; PDE 6C antibody; PDE6 alpha prime antibody; PDE6 alpha' antibody; PDE6C antibody; PDE6C_HUMAN antibody; PDEA2 antibody; Phosphodiesterase 6C cGMP specific cone alpha prime antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Cone cGMP-specific 3\',5\'-cyclic phosphodiesterase subunit alpha\' protein (285-451AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，PDE6C Antibody (CSB-PA23139A0Rb)，的标记方式是Non-conjugated。对于PDE6C Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA23139B0Rb PDE6C Antibody, HRP conjugated ELISA
    FITC CSB-PA23139C0Rb PDE6C Antibody, FITC conjugated
    Biotin CSB-PA23139D0Rb PDE6C Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, WB, IF
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:5000
    IF 1:50-1:200
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

产品评价

靶点详情

  • 功能：
    As cone-specific cGMP phosphodiesterase, it plays an essential role in light detection and cone phototransduction by rapidly decreasing intracellular levels of cGMP.
  • 基因功能参考文献：
    1. This study reveals two general mechanisms of missense PDE6C mutations underlying retinal diseases: (a) inability of AIPL1 to fold mutant PDE6C proteins leading to complete catalytic inactivity and (b) failure of P gamma regulatory subunit to serve as co-chaperone with AIPL1 in folding of mutant PDE6C. PMID: 28583373
    2. A novel homozygous PDE6C mutation was identified as the cause of ACHM. In addition, we identified an OPN1SW mutation in the sibling with complete achromatopsia. PMID: 25605338
    3. Expression of PDE6 in rod photoreceptors show that the cone PDE6 isoform is responsible for the difference in light adaptation between rods and cones. PMID: 26085644
    4. The majority (n = 12) of patients were either homozygotes or compound heterozygotes for known achromatopsia alleles, two in CNGB3 (p.T383fsX and p.T296YfsX9) and three in CNGA3 (p.R283Q, p.R427C and p.L527R). PMID: 23362848
    5. Missense mutations, nonsense mutations, splice mutations, and small deletions and insertions in the affected genes cause achromatopsia. PMID: 21267001
    6. Eleven different PDE6C mutations were found including two nonsense mutations, three mutations affecting transcript splicing as shown by minigene assays, one 1 bp-insertion and five missense mutations PMID: 21127010
    7. Homozygosity mapping reveals PDE6C mutations in patients with early-onset cone photoreceptor disorders. PMID: 19615668
    8. analysis of human cone phosphodiesterase-6 ectopically expressed in Xenopus laevis rods PMID: 19801642
    9. the identification of mutations in the PDE6C gene encoding the catalytic subunit of the cone photoreceptor phosphodiesterase as a cause of autosomal recessive achromatopsia was reported. PMID: 19887631

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  • 相关疾病：
    Cone dystrophy 4 (COD4); Achromatopsia 5 (ACHM5)
  • 亚细胞定位：
    Cell membrane; Lipid-anchor; Cytoplasmic side.
  • 蛋白家族：
    Cyclic nucleotide phosphodiesterase family
  • 数据库链接：

    HGNC: 8787

    OMIM: 600827

    KEGG: hsa:5146

    STRING: 9606.ENSP00000360502

    UniGene: Hs.658121