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Recombinant Human Tricarboxylate transport protein, mitochondrial (SLC25A1)

  • 中文名稱:
    人SLC25A1重組蛋白
  • 貨號:
    CSB-CF021475HU
  • 規格:
  • 來源:
    in vitro E.coli expression system
  • 其他:

產品詳情

  • 基因名:
  • Uniprot No.:
  • 別名:
    Citrate transport protein; CTP; mitochondrial; SLC20A3; Slc25a1; solute carrier family 20 (mitochondrial citrate transporter), member 3; solute carrier family 25 (mitochondrial carrier, citrate transporter), member 1; Solute carrier family 25 member 1; Tricarboxylate carrier protein; Tricarboxylate transport protein; tricarboxylate transport protein, mitochondrial; TXTP_HUMAN
  • 種屬:
    Homo sapiens (Human)
  • 蛋白長度:
    Full Length of Mature Protein
  • 表達區域:
    14-311
  • 氨基酸序列
    APASGKAKLTHPGKAILAGGLAGGIEICITFPTEYVKTQLQLDERSHPPRYRGIGDCVRQ TVRSHGVLGLYRGLSSLLYGSIPKAAVRFGMFEFLSNHMRDAQGRLDSTRGLLCGLGAGV AEAVVVVCPMETIKVKFIHDQTSPNPKYRGFFHGVREIVREQGLKGTYQGLTATVLKQGS NQAIRFFVMTSLRNWYRGDNPNKPMNPLITGVFGAIAGAASVFGNTPLDVIKTRMQGLEA HKYRNTWDCGLQILKKEGLKAFYKGTVPRLGRVCLDVAIVFVIYDEVVKLLNKVWKTD
    Note: The complete sequence may include tag sequence, target protein sequence, linker sequence and extra sequence that is translated with the protein sequence for the purpose(s) of secretion, stability, solubility, etc.
    If the exact amino acid sequence of this recombinant protein is critical to your application, please explicitly request the full and complete sequence of this protein before ordering.
  • 蛋白標簽:
    10xHis-SUMO-tag
  • 產品提供形式:
    Liquid or Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 緩沖液:
    Lyophilized from Tris/PBS-based buffer, 6% Trehalose, pH 8.0
  • 儲存條件:
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保質期:
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 貨期:
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 注意事項:
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet & COA:
    Please contact us to get it.

產品評價

靶點詳情

  • 功能:
    Citrate transporter that mediates the exchange of mitochondrial citrate for cytosolic malate. Also able to mediate the exchange of citrate for isocitrate, phosphoenolpyruvate, cis- but not trans-aconitate and to a lesser extend maleate and succinate. Important for the bioenergetics of hepatic cells as it provides a carbon source for fatty acid and sterol biosyntheses, and NAD(+) for the glycolytic pathway. Required for proper neuromuscular junction formation.
  • 基因功能參考文獻:
    1. SLC25A1 and ACLY upregulation suggests that metabolic reprogramming in Behcet's syndrome involves the citrate pathway dysregulation. PMID: 30050389
    2. tested the expression specificity of the Cochlin-tomoprotein by testing blood and CSF samples. The concentration was below the detection limit (0.2 ng/ml) in 38 of the 40 blood, and 14 of the 19 CSF samples PMID: 29377917
    3. Pathogenic mutations of the human mitochondrial citrate carrier SLC25A1 lead to impaired citrate export required for lipid, dolichol, ubiquinone and sterol synthesis. PMID: 29031613
    4. this study shows increased expression of SLC25A1 gene in cells from children with Down syndrome PMID: 27502741
    5. Altered metabolism in 22qDS reflected a critical role for the haploinsufficiency of the mitochondrial citrate transporter SLC25A1, further enhanced by HIF-1alpha, MYC, and metabolite controls. PMID: 26221035
    6. we report for the first time on a patient with a genetically confirmed diagnosis of SLC25A1 deficiency and treatment with either malate or citrate PMID: 24687295
    7. SLC25A1 has a key role in TNF-alpha and IFNgamma induced inflammation and is induced at the transcriptional level by these two inflammation mediators cytokines. PMID: 25072865
    8. We report for the first time a patient with a mitochondrial citrate carrier deficiency. Our data support a role for citric acid cycle defects in agenesis of corpus callosum PMID: 23393310
    9. Compares and contrasts all the known human SLC25A* genes and includes functional information. PMID: 23266187
    10. Deficiency in SLC25A1, encoding the mitochondrial citrate carrier, causes combined D-2- and L-2-hydroxyglutaric aciduria. PMID: 23561848
    11. The mitochondrial citrate carrier (CIC) is present and regulates insulin secretion by human male gamete. PMID: 22355067
    12. muscular symptoms of CTP deficiency respond to creatine supplementation PMID: 21660517
    13. The mitochondrial citrate carrier: a new player in inflammation PMID: 21787310
    14. The results of molecular cloning of a citrate transporter from human normal prostate epithelial PNT2-C2 cells, is reported. PMID: 20448665
    15. Results suggest an evolutionary conserved role for Sea/SLC25A1 in the regulation of chromosome integrity. PMID: 19654186
    16. These results show that methylation, histone acetylation and Sp1 are important in the transcriptional regulation of the CIC proximal promoter. PMID: 18706393
    17. These results show that FOXA plays a role in the transcriptional regulation of CIC and in insulin secretion. PMID: 19445897
    18. CIC silencer activity extends over 26 bp (-595/-569), which specifically bind a protein ZNF224 present in HepG2 cell nuclear extracts. PMID: 19505435

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  • 相關疾病:
    Combined D-2- and L-2-hydroxyglutaric aciduria (D2L2AD)
  • 亞細胞定位:
    Mitochondrion inner membrane; Multi-pass membrane protein.
  • 蛋白家族:
    Mitochondrial carrier (TC 2.A.29) family
  • 數據庫鏈接:

    HGNC: 10979

    OMIM: 190315

    KEGG: hsa:6576

    STRING: 9606.ENSP00000215882

    UniGene: Hs.111024



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