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  4. ALAS1 Recombinant Monoclonal Antibody

ALAS1 Recombinant Monoclonal Antibody

  • 货号：
    CSB-RA266893A0HU
  • 规格：
    ￥1320
  • 图片：
    • Western Blot
      Positive WB detected in: Hela whole cell lysate, HepG2 whole cell lysate, Raji whole cell lysate, PC-3 whole cell lysate, A549 whole cell lysate, MCF-7 whole cell lysate
      All lanes: ALAS1 antibody at 1:1500
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 71, 13 kDa
      Observed band size: 71 kDa
  • 其他：

产品详情

  • 产品描述：

    The recombinant ALAS1 antibody is a monoclonal antibody molecule expressed by using recombinant DNA and protein engineering technology to clone the genes encoding the ALAS1 antibody into a plasma vector and then by transfecting the vector clone into the appropriate recipient mammalian cells for production. It was purified using affinity-chromatography. And it shows reactivity with ALAS1 protein from Human. This recombinant ALAS1 antibody can be used in the ELISA, WB.

    ALAS1 is a mitochondrial enzyme that catalyzes the condensation of glycine and succinyl-CoA generating 5-aminolevulinic acid. As a rate-limiting enzyme of the heme biosynthetic pathway, ALAS1 provides a basal level of heme in nonerythropoietic cells for cytochromes and other hemoproteins. ALAS1 is tightly controlled to adjust to hepatocytes' metabolic demands. ALAS1 deficiency causes embryonic mortality in mice, showing that it is required for early embryogenesis.

  • Uniprot No.：
    P13196
  • 基因名：
    ALAS1
  • 别名：
    5-aminolevulinate synthase, nonspecific, mitochondrial (ALAS-H) (EC 2.3.1.37) (5-aminolevulinic acid synthase 1) (Delta-ALA synthase 1) (Delta-aminolevulinate synthase 1), ALAS1, ALAS3 ALASH
  • 反应种属：
    Human
  • 免疫原：
    A synthesized peptide derived from human Alas1
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 克隆类型：
    Monoclonal
  • 抗体亚型：
    Rabbit IgG
  • 纯化方式：
    Affinity-chromatography
  • 克隆号：
    2A9
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Rabbit IgG in phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, WB
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:5000
  • 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

  • 基因功能参考文献：
    1. The mutation in CLPX inactivates its ATPase activity, resulting in coassembly of mutant and WT protomers to form an enzyme with reduced activity. The presence of low-activity CLPX increases the posttranslational stability of ALAS, causing increased ALAS protein and ALA levels, leading to abnormal accumulation of PPIX. PMID: 28874591
    2. the heme-binding site in the N-terminal region of the mature ALAS1 protein is also necessary for the heme-dependent oxidation of ALAS1. PMID: 27496948
    3. ALAS1 mRNA and activity were elevated approximately ~3- and 5-fold, and HMB synthase activity was approximately half-normal (~42%) PMID: 26062020
    4. The -853T variant functions as an enhancer in the presence of estrogen and speculates that the -1253A variant reduces transcription activity. PMID: 19656447
    5. These results indicate that ALAS1 is a novel NR5A-target gene and participates in steroid hormone production. PMID: 23024262
    6. Lon peptidase 1 (LONP1)-dependent breakdown of mitochondrial 5-aminolevulinic acid synthase protein by heme in human liver cells. PMID: 21659532
    7. REVIEW:active site and mechanistic analysis, protein folding, structure and function. PMID: 11929042
    8. REVIEW: mechanisms involving ALAS deficiency, point mutations, post translational processing, and complex formation with succinyl CoA synthetase subunit B in the pathogenesis of hereditary sideroblastic anemia. PMID: 11929048
    9. ALAS expression is regulated by AP-1 complex through sequestration of cAMP-response element protein (CRE)-binding protein (CBP) coactivator in human cells PMID: 12433930
    10. ALAS gene expression is regulated by Hepatic nuclear factor 3 and nuclear factor 1 PMID: 15123725
    11. First described frameshift ALAS2 mutation, CD506-507 (-C). PMID: 15477213
    12. in the liver of Acute liver failure patients, there may be an increase in free heme concentration which down-regulating ALAS1 gene expression PMID: 15547665
    13. Alternative splicing of human ALAS1 generates two mRNAs with different 5'-UTRs: a major one, where exon 1B is omitted, and a minor form containing exon 1B. PMID: 15710391
    14. 5-aminolevulinate synthase gene repression by the potent tumor promoter, TPA, involves multiple signal transduction pathways PMID: 15797241
    15. Expression of candidate genes HPRT1 and ALAS1 in malignant and non-malignant prostate tissue samples after microdissection. PMID: 17628775
    16. Differential regulation of human ALAS1 mRNA and protein levels by heme and cobalt protoporphyrin. PMID: 18719978
    17. In this study, we show significant reductions of the rate-limiting enzymes involved in heme biosynthesis, ALAS1 in the postmortem cortex of Alzheimer's disease subjects, providing additional evidence of abnormal heme homeostasis in Alzheimer's disease. PMID: 19477221

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  • 亚细胞定位：
    Mitochondrion matrix.
  • 蛋白家族：
    Class-II pyridoxal-phosphate-dependent aminotransferase family
  • 数据库链接：

    HGNC: 396

    OMIM: 125290

    KEGG: hsa:211

    STRING: 9606.ENSP00000309259

    UniGene: Hs.476308