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Human Glycyl-tRNA synthetase(GARS) ELISA kit

  • 中文名稱:
    人甘氨酰-tRNA合成酶(GARS)酶聯免疫試劑盒
  • 貨號:
    CSB-EL009262HU
  • 規格:
    96T/48T
  • 價格:
    ¥3600/¥2500
  • 其他:

產品詳情

  • 產品描述:
    人甘氨酰-tRNA合成酶(GARS)酶聯免疫試劑盒(CSB-EL009262HU)為雙抗夾心法ELISA試劑盒,定量檢測血清、血漿、組織勻漿、細胞裂解物樣本中的GARS含量。GARS(甘氨酰-tRNA合成酶)是一種重要的蛋白質合成酶,其基因突變可能導致多種神經退行性疾病。研究GARS的靶點,旨在揭示其與疾病的關系,為疾病的治療提供新思路。研究機制主要涉及GARS在蛋白質合成中的作用,以及其突變如何影響細胞功能。試劑盒檢測范圍為31.25 pg/mL-2000 pg/mL,適用于細胞生物學、分子機制研究及疾病模型分析,例如探究藥物干預、基因編輯或病理條件下GARS的表達調控及其功能變化,為蛋白質合成異常相關研究提供定量工具。本品僅用于科研,不用于臨床診斷,產品具體參數及操作步驟詳見產品說明書。
  • 別名:
    GARS1 ELISA Kit; GARSGlycine--tRNA ligase ELISA Kit; EC 6.1.1.14 ELISA Kit; Diadenosine tetraphosphate synthetase ELISA Kit; Ap4A synthetase ELISA Kit; EC 2.7.7.- ELISA Kit; Glycyl-tRNA synthetase ELISA Kit; GlyRS ELISA Kit; Glycyl-tRNA synthetase 1 ELISA Kit
  • 縮寫:
    GARS
  • Uniprot No.:
  • 種屬:
    Homo sapiens (Human)
  • 樣本類型:
    serum, plasma, tissue homogenates, cell lysates
  • 檢測范圍:
    31.25 pg/mL-2000 pg/mL
  • 靈敏度:
    7.81 pg/mL
  • 反應時間:
    1-5h
  • 樣本體積:
    50-100ul
  • 檢測波長:
    450 nm
  • 研究領域:
    Metabolism
  • 測定原理:
    quantitative
  • 測定方法:
    Sandwich
  • 精密度:
    Intra-assay Precision (Precision within an assay): CV%<8%
    Three samples of known concentration were tested twenty times on one plate to assess.
    Inter-assay Precision (Precision between assays): CV%<10%
    Three samples of known concentration were tested in twenty assays to assess.
  • 線性度:
    To assess the linearity of the assay, samples were spiked with high concentrations of human GARS in various matrices and diluted with the Sample Diluent to produce samples with values within the dynamic range of the assay.
    SampleSerum(n=4)
    1:1Average %96
    Range %91-99
    1:2Average %94
    Range %90*98
    1:4Average %95
    Range %90-103
    1:8Average %95
    Range %89-105
  • 回收率:
    The recovery of human GARS spiked to levels throughout the range of the assay in various matrices was evaluated. Samples were diluted prior to assay as directed in the Sample Preparation section.
    Sample TypeAverage % RecoveryRange
    Serum (n=5) 8984-92
    EDTA plasma (n=4)9185-96
  • 標準曲線:
    These standard curves are provided for demonstration only. A standard curve should be generated for each set of samples assayed.
    pg/mlOD1OD2AverageCorrected
    20002.121 2.097 2.109 2.006
    10001.867 1.848 1.858 1.755
    5001.538 1.527 1.533 1.430
    2501.107 1.101 1.104 1.001
    1250.701 0.699 0.700 0.597
    62.50.497 0.492 0.495 0.392
    31.250.283 0.275 0.279 0.176
    00.105 0.101 0.103
  • 數據處理:
  • 貨期:
    3-5 working days

產品評價

靶點詳情

  • 功能:
    Catalyzes the ATP-dependent ligation of glycine to the 3'-end of its cognate tRNA, via the formation of an aminoacyl-adenylate intermediate (Gly-AMP). Also produces diadenosine tetraphosphate (Ap4A), a universal pleiotropic signaling molecule needed for cell regulation pathways, by direct condensation of 2 ATPs. Thereby, may play a special role in Ap4A homeostasis.
  • 基因功能參考文獻:
    1. In support of GARS variant pathogenicity, our patient shows striking phenotypic overlap with other patients having ARS-related recessive diseases; this observation is consistent with the essential function of GARS in both cellular locations. In summary, our clinical, genetic, and functional analyses expand the phenotypic spectrum associated with GARS variants PMID: 28675565
    2. In this Chinese Han population a novel Charcot-Marie-Tooth disease-associated gene mutations of GARS (c.794C>T) was discovered. PMID: 27862672
    3. At the active site, a glycyl-AMP molecule is synthesized and is waiting for the transfer of the glycyl moiety to occur. PMID: 27261259
    4. GlyRS functions as a chaperone that critically supports neddylation. PMID: 27348078
    5. Data indicate that dimerization is required for the dominant neurotoxicity of disease-associated GARS mutations and provide a rapid, tractable model for studying newly identified GARS variants for a role in human disease. PMID: 27008886
    6. one of the mRNAs isoforms tightly controls expression and localization of human GARS. PMID: 26327585
    7. This study reports two crystal structures of human GlyRS variants, in the free form and in complex with tRNA(Gly) respectively, and reveal new aspects of the glycylation mechanism. PMID: 26797133
    8. GARS mutations are an uncommon cause of Charcot-Marie-Tooth Disease (CMT) in Taiwan. The p.Asp146Tyr and p.Met238Arg mutations are associated with early-onset axonal CMT. PMID: 26244500
    9. Our findings suggest that mutant GlyRS gains access to ectopic sub-compartments of the motor neuron, providing a possible explanation for the selective neuropathology caused by mutations in a widely expressed gene. PMID: 25972375
    10. Expression of three CMT-mutant GARS proteins in Drosophila induces defects in motor performance and motor and sensory neuron morphology, and shortens lifespan. PMID: 26138142
    11. The c.999G>T mutation is a novel mutation of the glycyl-tRNA synthetase gene that has not been previously reported. The phenotype of this family is Charcot-Marie-Tooth disease type 2D, which is first reported in Chinese population. PMID: 26000875
    12. we propose that the disease-causing L129P mutant of glycyl-tRNA synthetase is linked to a distribution defect in peripheral nerves in vivo. PMID: 25218976
    13. our data indicate that impaired function is a key component of GARS-mediated CMT disease and emphasize the need for careful genetic and functional evaluation before implicating a variant in disease onset. PMID: 25168514
    14. This study presents genetic evidence for common mutant-specific interactions between two CMT-associated aminoacyl-tRNA synthetases, lending support for a shared mechanism responsible for the synthetase-induced peripheral neuropathies. PMID: 24807208
    15. We developed an ELISA to detect anti-glycyl-tRNA synthetase by using recombinant protein PMID: 24508626
    16. Report crystal structures of wild type and mutant GlyRS in complex with tRNA and with small substrates and describe the molecular details of enzymatic recognition of the key tRNA identity elements in the acceptor stem and the anticodon loop. PMID: 24898252
    17. we believe that these two novel GARS mutations are the underlying causes of the distal hereditary motor neuropathy type V phenotype PMID: 23279345
    18. GRS bound to different ERK-activated tumor cells, and released phosphatase 2A (PP2A) from CDH6. PMID: 22345558
    19. missense mutations of Gars may cause some loss of function, the dominant neuropathy phenotype observed in mice is caused by a dose-dependent gain of function that is not mitigated by over-expression of functional wild-type protein. PMID: 22144914
    20. No pathogenic mutations were found, excluding the role of GARS gene as a possible factor in the aetiology of Hirayama disease in this cohort PMID: 19412816
    21. GARS mutation is a rare cause of Charcot-Marie-Tooth neuropathy among Japanese patients. PMID: 19329989
    22. Four disease-associated missense mutations in the glycyl tRNA synthetase gene in families with Charcot-Marie-Tooth disease type 2D and distal spinal muscular atrophy type V PMID: 12690580
    23. A novel heterozygous missense GARS gene mutation (D500N) was identified in members of a family affected byCharcot-Marie-Tooth type 2D. PMID: 16534118
    24. We screened 100 patients with inherited and sporadic lower motor neuron degeneration and identified three novel missense mutations in the glycyl-tRNA synthetase (GARS) gene. PMID: 17101916
    25. The crystal belonged to space group P4(3)2(1)2 or its enantiomorphic space group P4(1)2(1)2, & diffracted X-rays to 3.0 A resolution. The asymmetric unit contained 1 GlyRS molecule & had a solvent content of 69%. PMID: 17142907
    26. Crystal structure of human wildtype and S581L-mutant glycyl-tRNA synthetase. PMID: 17544401
    27. The structure of wild type and Charcot-Marie-Tooth-causing mutant of homodimeric GlyRS are reported. PMID: 17545306
    28. Charcot-Marie-Tooth (CMT) disease-causing mutations of glycine-tRNA synthetase share a common defect in localization which may be connected to a change in surfaces at the dimer interface, and may cause a dominant axonal form of CMT (type 2D). PMID: 17595294
    29. we present a comparison between the crystal structures of the eubacterial Escherichia coli and the human tRNA(Gly) acceptor stem microhelices and their surrounding hydration patterns. PMID: 18275849
    30. human glycyl-tRNA synthetase has a role in Ap4A homeostasis PMID: 19710017

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  • 相關疾病:
    Charcot-Marie-Tooth disease 2D (CMT2D); Neuronopathy, distal hereditary motor, 5A (HMN5A)
  • 亞細胞定位:
    Cytoplasm. Cell projection, axon. Secreted. Secreted, extracellular exosome.; [Isoform 1]: Mitochondrion. Cytoplasm.; [Isoform 2]: Cytoplasm. Cell projection, axon.
  • 蛋白家族:
    Class-II aminoacyl-tRNA synthetase family
  • 組織特異性:
    Widely expressed, including in brain and spinal cord.; [Isoform 2]: Expressed in brain, spinal cord, muscle, heart and spleen.; [Isoform 1]: Expressed in brain, spinal cord, muscle, heart, spleen and liver.
  • 數據庫鏈接:

    HGNC: 4162

    OMIM: 600287

    KEGG: hsa:2617

    STRING: 9606.ENSP00000373918

    UniGene: Hs.404321



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