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  4. ABCD3 Recombinant Monoclonal Antibody

ABCD3 Recombinant Monoclonal Antibody

  • 中文名稱：
    ABCD3 Recombinant Monoclonal Antibody
  • 貨號：
    CSB-RA843022A0HU
  • 規格：
    ￥1320
  • 圖片：
    • Western Blot
      Positive WB detected in:Mouse brain tissue lysate(30μg), Rat brain tissue lysate(30μg)
      All lanes: ABCD3 antibody at 1:1000
      Secondary
      Goat polyclonal to rabbit IgG at 1/40000 dilution
      Predicted band size: 75 kDa
      Observed band size:70 kDa
      Exposure time：30s
    • IHC image of CSB-RA843022A0HU diluted at 1:100 and staining in paraffin-embedded human liver cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB.
    • Overlay Peak curve showing HepG2 cells stained with CSB-RA843022A0HU (red line) at 1:100. The cells were fixed in 4% formaldehyde and permeated by 0.2% TritonX-100 for?10min. Then 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (1ug/1*106cells) for 45min at 4℃. The secondary antibody used was FITC-conjugated goat anti-rabbit IgG (H+L) at 1/200 dilution for 35min at 4℃.Control antibody (green line) was Rabbit IgG (1ug/1*106cells) used under the same conditions. Acquisition of >10,000 events was performed.
  • 其他：

產品詳情

  • Uniprot No.：
  • 基因名：
    ABCD3
  • 別名：
    ATP-binding cassette sub-family D member 3 (70 kDa peroxisomal membrane protein) (PMP70), ABCD3, PMP70 PXMP1
  • 反應種屬：
    Human, Mouse, Rat
  • 免疫原：
    A synthesized peptide derived from Human ABCD3 protein
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    Non-conjugated
  • 克隆類型：
    Monoclonal
  • 抗體亞型：
    Rabbit IgG
  • 純化方式：
    Affinity-chromatography
  • 克隆號：
    16H5
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Rabbit IgG in 10mM phosphate buffered saline , pH 7.4, 150mM sodium chloride, 0.05% BSA, 0.02% sodium azide and 50% glycerol.
  • 產品提供形式：
    Liquid
  • 應用范圍：
    ELISA, WB, IHC, FC
  • 推薦稀釋比：
    Application Recommended Dilution
    WB 1:500-1:5000
    IHC 1:50-1:200
    FC 1:20-1:200
  • Protocols：
  • 儲存條件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期：
    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.
  • 用途：
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

  • 功能：
    Broad substrate specificity ATP-dependent transporter of the ATP-binding cassette (ABC) family that catalyzes the transport of long-chain fatty acids (LCFA)-CoA, dicarboxylic acids-CoA, long-branched-chain fatty acids-CoA and bile acids from the cytosol to the peroxisome lumen for beta-oxydation. Has fatty acyl-CoA thioesterase and ATPase activities. Probably hydrolyzes fatty acyl-CoAs into free fatty acids prior to their ATP-dependent transport into peroxisomes. Thus, play a role in regulation of LCFAs and energy metabolism namely, in the degradation and biosynthesis of fatty acids by beta-oxidation.
  • 基因功能參考文獻：
    1. ABCD1 and ABCD2 are involved in the transport of long and very long chain fatty acids (VLCFA) or their CoA-derivatives into peroxisomes with different substrate specificities, while ABCD3 is involved in the transport of branched chain acyl-CoA into peroxisomes.ABCD4 is deduced to take part in the transport of vitamin B12 from lysosomes into the cytosol. PMID: 27766264
    2. Data show that ACBD3 can recruit PI4KB to model membranes as well as redirect PI4KB to cellular membranes where it is not naturally found. Also, results show that ACBD3 regulates the enzymatic activity of PI4KB kinase through membrane recruitment rather than allostery. PMID: 27009356
    3. The N-terminal motif of PMP70 suppresses cotranslational targeting to the endoplasmic reticulum. PMID: 26711236
    4. Increased ABCD3 expression correlates with Gleason Score. PMID: 25802834
    5. Deficiency of peroxisomal ABCD3 resulted in bile acid biosynthesis defect. PMID: 25168382
    6. We postulate a role for human ABCD3 in the oxidation of dicarboxylic acids and a role in buffering fatty acids that are overflowing from the mitochondrial beta-oxidation system PMID: 24333844
    7. investigation of organelle-targeting properties of N-terminal portions of peroxisomal PMP70; amino acid sequence and domain structure of human form discussed PMID: 20007743
    8. Testosterone metabolites did not alter expression of ABCD3 mRNA in fibroblasts from X-linked adrenoleukodystrophy patients. PMID: 17602313
    9. ALDRP interacts with PMP70. This interaction occurs via the ALDRP C-terminus [374-740] and the PMP70 C-terminus [338-659]. This interaction was demonstrated using human PMP70 and mouse ALDRP. PMID: 10551832
    10. ALDP interacts with PMP70. This interaction occurs via the C-terminus of ALDP [361-745] and the C-terminus of PMP70 [338-659]. ALDP mutations P484R and R591Q abolish the interaction. PMID: 10551832
    11. PMP70 (ABCD3) homodimerizes via the carboxy terminal half [338-659]. PMID: 10551832

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  • 相關疾病：
    Congenital bile acid synthesis defect 5 (CBAS5)
  • 亞細胞定位：
    Peroxisome membrane; Multi-pass membrane protein.
  • 蛋白家族：
    ABC transporter superfamily, ABCD family, Peroxisomal fatty acyl CoA transporter (TC 3.A.1.203) subfamily
  • 數據庫鏈接：

    HGNC: 67

    OMIM: 170995

    KEGG: hsa:5825

    STRING: 9606.ENSP00000359233

    UniGene: Hs.700576



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