在线日韩日本国产亚洲丨少妇伦子伦情品无吗丨欧美性猛交xxxx免费看蜜桃丨精品人妻系列无码一区二区三区丨亚洲精品无码不卡在线播放

Your Good Partner in Biology Research

GARS Antibody, Biotin conjugated

  • 中文名稱:
    GARS兔多克隆抗體, Biotin偶聯
  • 貨號:
    CSB-PA009262ED01HU
  • 規格:
    ¥880
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) GARS Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    GARS
  • 別名:
    GARS1 antibody; GARSGlycine--tRNA ligase antibody; EC 6.1.1.14 antibody; Diadenosine tetraphosphate synthetase antibody; Ap4A synthetase antibody; EC 2.7.7.- antibody; Glycyl-tRNA synthetase antibody; GlyRS antibody; Glycyl-tRNA synthetase 1 antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human
  • 免疫原:
    Recombinant Human Glycine--tRNA ligase protein (1-389AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標記方式:
    Biotin
  • 克隆類型:
    Polyclonal
  • 抗體亞型:
    IgG
  • 純化方式:
    >95%, Protein G purified
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 產品提供形式:
    Liquid
  • 應用范圍:
    ELISA
  • 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

    顯示更多

    收起更多

  • 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



主站蜘蛛池模板: 亚洲国产综合在线区尤物| 国内外精品激情刺激在线| 亚洲国产成人超a在线播放| 激情av无码后入| 波多野结衣不打码视频| 国产永久免费观看视频| av在线亚洲欧洲日产一区二区 | 日韩av高清无码| 久久国产福利一区二区| 欧美亚洲熟妇一区二区三区| 国产精品av在线| 国产动作大片中文字幕| 午夜福利18以下勿进免费| 国产成年女人毛片80s网站| 国产成人无码a区精油按摩| 久久久久无码中| 日本亚洲欧美日韩国产ay| 久久精品视频在线看| 欧美精品videosex性欧美| 女同性av片在线观看免费网站| 国产成人无码精品久久涩吧| 精品国产va久久久久久久冰 | 精品无人区卡一卡二卡三乱码 | 久久亚洲精品无码网站| 狠狠躁夜夜躁人人爽天天bl| 无码视频在线观看| 日韩无码电影| 日韩欧美亚洲综合久久影院d3| 色偷偷女人的天堂亚洲网| 一本大道无码人妻精品专区| 青青草国产精品日韩欧美| 亚洲精品一区二区国产精华液| 日韩高清国产一区在线| 无码中文av有码中文av| 99九九99九九视频精品| 在线观看无码av免费不卡软件| 国自产拍偷拍精品啪啪一区二区| 日本高清www午色夜在线视频| 裸体丰满白嫩大尺度尤物| 俺去俺来也在线www色官网| s级爆乳玩具酱国产vip皮裤|