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ALAS1 Recombinant Monoclonal Antibody

  • 中文名稱：
    ALAS1重組抗體
  • 貨號：
    CSB-RA266893A0HU
  • 規(guī)格：
    ￥1320
  • 圖片：
    • 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：

產(chǎn)品詳情

  • 產(chǎn)品描述：
    CSB-RA266893A0HU ALAS1重組單克隆抗體是針對δ-氨基乙酰丙酸合酶1（ALAS1）的高特異性科研工具，該靶標(biāo)是血紅素生物合成的限速酶，在細(xì)胞能量代謝和氧轉(zhuǎn)運過程中發(fā)揮關(guān)鍵作用。本抗體通過重組技術(shù)制備，具有優(yōu)異的批次一致性和低交叉反應(yīng)性，經(jīng)ELISA與Western Blot（WB）嚴(yán)格驗證，在WB實驗中推薦使用1:500至1:5000的寬范圍稀釋度，可清晰檢測內(nèi)源性ALAS1蛋白表達(dá)。其應(yīng)用場景涵蓋血紅素合成通路機制研究、鐵代謝相關(guān)疾病模型分析，以及藥物干預(yù)對線粒體功能影響的分子水平評估，適用于肝細(xì)胞、紅細(xì)胞前體細(xì)胞等樣本的蛋白質(zhì)表達(dá)研究。該產(chǎn)品為科研人員探索ALAS1在遺傳性卟啉癥、貧血等病理過程中的作用提供了可靠工具，實驗結(jié)果具備高重復(fù)性與靈敏度，滿足基礎(chǔ)研究中對靶標(biāo)蛋白精準(zhǔn)檢測的需求。
  • Uniprot No.：
  • 基因名：
    ALAS1
  • 別名：
    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
  • 反應(yīng)種屬：
    Human
  • 免疫原：
    A synthesized peptide derived from human Alas1
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標(biāo)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：
    2A9
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Rabbit IgG in phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
  • 產(chǎn)品提供形式：
    Liquid
  • 應(yīng)用范圍：
    ELISA, WB
  • 推薦稀釋比：
    Application Recommended Dilution
    WB 1:500-1:5000
  • 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(chǎn)品評價

靶點詳情

  • 基因功能參考文獻(xiàn)：
    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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  • 亞細(xì)胞定位：
    Mitochondrion matrix.
  • 蛋白家族：
    Class-II pyridoxal-phosphate-dependent aminotransferase family
  • 數(shù)據(jù)庫鏈接：

    HGNC: 396

    OMIM: 125290

    KEGG: hsa:211

    STRING: 9606.ENSP00000309259

    UniGene: Hs.476308