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ALAS1 Antibody

  • 货号：
    CSB-PA929439
  • 规格：
    ￥1100
  • 图片：
    • The image on the left is immunohistochemistry of paraffin-embedded Human breast cancer tissue using CSB-PA929439(ALAS1 Antibody) at dilution 1/30, on the right is treated with fusion protein. (Original magnification: ×200)
    • The image on the left is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using CSB-PA929439(ALAS1 Antibody) at dilution 1/30, on the right is treated with fusion protein. (Original magnification: ×200)
  • 其他：

产品详情

  • Uniprot No.：
    P13196
  • 基因名：
    ALAS1
  • 别名：
    5 aminolevulinate synthase antibody; 5 aminolevulinate synthase nonspecific mitochondrial antibody; 5 aminolevulinic acid synthase antibody; 5-aminolevulinate synthase antibody; 5-aminolevulinic acid synthase 1 antibody; Alas 1 antibody; ALAS 3 antibody; ALAS antibody; ALAS H antibody; ALAS HOUSEKEEPING TYPE antibody; ALAS N antibody; ALAS-H antibody; alaS1 antibody; ALAS3 antibody; ALASH antibody; Aminolevulinate delta synthase 1 antibody; Aminolevulinic acid synthase 1 antibody; Delta ALA synthetase antibody; Delta aminolevulinate synthase antibody; Delta-ALA synthase 1 antibody; Delta-aminolevulinate synthase 1 antibody; HEM1_HUMAN antibody; MIG 4 antibody; MIG4 antibody; Migration inducing protein 4 antibody; mitochondrial antibody; nonspecific antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Fusion protein of Human ALAS1
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen affinity purification
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,IHC
  • 推荐稀释比：
    Application Recommended Dilution
    ELISA 1:2000-1:5000
    IHC 1:25-1:100
  • 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