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ASL Antibody

  • 货号：
    CSB-PA002213LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Immunohistochemistry of paraffin-embedded human liver cancer using CSB-PA002213LA01HU at dilution of 1:100
    • Immunohistochemistry of paraffin-embedded human liver tissue using CSB-PA002213LA01HU at dilution of 1:100
    • Immunofluorescence staining of Hela cells with CSB-PA002213LA01HU at 1:100, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) ASL Polyclonal antibody
  • Uniprot No.：
    P04424
  • 基因名：
  • 别名：
    Argininosuccinase antibody; Argininosuccinate lyase antibody; Arginosuccinase antibody; ARLY_HUMAN antibody; ASAL antibody; ASL antibody; EC 4.3.2.1 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Argininosuccinate lyase protein (2-300AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，ASL Antibody (CSB-PA002213LA01HU)，的标记方式是Non-conjugated。对于ASL Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA002213LB01HU ASL Antibody, HRP conjugated ELISA
    FITC CSB-PA002213LC01HU ASL Antibody, FITC conjugated
    Biotin CSB-PA002213LD01HU ASL Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, IHC, IF
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:20-1:200
    IF 1:50-1:200
  • 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 remaining five patients were diagnosed with neonatal intrahepatic cholestasis due to citrin deficiency, and have respectively carried mutations of the SLC25A13 gene including [c.851-854delGTAT+c.851-854delGTAT], [c.851-854delGTAT+IVS6+5G>A], [c.851-854delGTAT+IVS16ins3kb], [c.851-854delGTAT+IVS6-11A>G] and [c.851-854delGTAT+c.1638-1660dup23] PMID: 28981931
    2. Overexpression of ASL may be a contributing factor in drug resistance for arginine deprivation therapy. PMID: 28035420
    3. ASL-targeting shRNA-induced growth inhibition is associated with decreased cyclin A2 expression and Nitric oxide content in colon cancer. PMID: 27840980
    4. the mechanism induced by ASL shRNA which occurred in human breast cancer may be attributed to a decrease in cyclin A2 and NO. PMID: 26397737
    5. The clinical and biochemical course in variant forms of ASL deficiency is associated with relevant residual levels of ASL activity as well as instability of mutant ASL proteins. PMID: 25778938
    6. Point mutation of ASS1, ASL and SLC25A13 is associated with citrullinemia. PMID: 24927999
    7. Data show that in patients with Argininosuccinate lyase deficiency, the ASl gene is subject to several mutations, the majority are missense; some more frequent then others. PMID: 24166829
    8. Our results suggest that ASL transcripts can contribute to the highly variable phenotype in ASA patients if expressed at high levels. PMID: 24136197
    9. Cox regression analysis showed that ASL is an independent prognostic marker for HCC. Therefore, reduced ASL expression may be a novel maker for poor prognosis in HCC patients PMID: 22531684
    10. analysis of mutant argininosuccinate lyase in argininosuccinic aciduria PMID: 21667091
    11. extent of protection of ASL and delta-crystallin at different ratios of alphaA-crystallin PMID: 20937351
    12. MDR analysis provided evidence of interaction between the genes for ASS1 and SLC25A13 on the risk of CL/P. PMID: 20739017
    13. Structural studies of the ASL frequently complementing allele Q286R suggest that the mutation may hinder a conformational change in the 280's loop (residues 270-290) and domain 3 that may be important for catalysis. PMID: 11747432
    14. Complementation can occur at the ASL locus between thermolabile mutants and stable mutants by stabilization of the active oligomeric form of the hybrid enzyme, which may be sufficiently stable for catalysis to occur. PMID: 11747433
    15. complete sequence of the human ASL gene and a complete ASL homologue on chromosome 22 PMID: 12384776
    16. argininosuccinic aciduria patients of different ethnic backgrounds who are characterized by residual activity of argininosuccinate lyase and who present with less severe clinical courses. PMID: 12408190
    17. This unique mutation causes an elongation of fifty amino acids in the C-terminal region of the ASL protein, and is likely related to a milder phenotype compared with previously reported mutations. PMID: 12512996
    18. a novel ASL pseudogene located in the centromeric region of chromosome 7, 14 novel mutations in the ASL gene, and a novel intronic polymorphism found in a cohort of Italian patients with argininosuccinic aciduria PMID: 17326097
    19. analysis of human missense argininosuccinate lyase mutations in yeast PMID: 19703900

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  • 相关疾病：
    Argininosuccinic aciduria (ARGINSA)
  • 蛋白家族：
    Lyase 1 family, Argininosuccinate lyase subfamily
  • 数据库链接：

    HGNC: 746

    OMIM: 207900

    KEGG: hsa:435

    STRING: 9606.ENSP00000307188

    UniGene: Hs.632015