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

  1. Home
  2. 抗體
  3. 多克隆抗體
  4. AFG3L2 Antibody

AFG3L2 Antibody

  • 中文名稱：
    AFG3L2兔多克隆抗體
  • 貨號：
    CSB-PA04005A0Rb
  • 規格：
    ￥440
  • 圖片：
    • Western blot
      All lanes: AFG3L2 antibody at 2µg/ml + EC109 whole cell lysate
      Secondary
      Goat polyclonal to rabbit IgG at 1/15000 dilution
      Predicted band size: 89 kDa
      Observed band size: 89 kDa
    • Immunohistochemistry of paraffin-embedded human prostate tissue using CSB-PA04005A0Rb at dilution of 1:100
  • 其他：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) AFG3L2 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    AFG3L2
  • 別名：
    AFG3 (ATPase family gene 3; yeast) like 2 antibody; AFG3 ATPase family gene 3 like 2 (yeast) antibody; AFG3 ATPase family gene 3 like 2 antibody; AFG3 like protein 2 antibody; AFG3-like protein 2 antibody; AFG32_HUMAN antibody; AFG3L2 antibody; ATPase family gene 3 like 2 antibody; ATPase family gene 3 yeast antibody; EC 3.4.24.- antibody; FLJ25993 antibody; Paraplegin like protein antibody; Paraplegin-like protein antibody; SCA28 antibody; Spinocerebellar ataxia 28 antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human
  • 免疫原：
    Recombinant Human AFG3-like protein 2 protein (550-759AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    Non-conjugated

    本頁面中的產品，AFG3L2 Antibody (CSB-PA04005A0Rb)，的標記方式是Non-conjugated。對于AFG3L2 Antibody，我們還提供其他標記。見下表：

    可提供標記
    標記方式 貨號 產品名稱 應用
    HRP CSB-PA04005B0Rb AFG3L2 Antibody, HRP conjugated ELISA
    FITC CSB-PA04005C0Rb AFG3L2 Antibody, FITC conjugated
    Biotin CSB-PA04005D0Rb AFG3L2 Antibody, Biotin conjugated ELISA
  • 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, WB, IHC
  • 推薦稀釋比：
    Application Recommended Dilution
    WB 1:1000-1:5000
    IHC 1:20-1:200
  • 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：
    ATP-dependent protease which is essential for axonal and neuron development. In neurons, mediates degradation of SMDT1/EMRE before its assembly with the uniporter complex, limiting the availability of SMDT1/EMRE for MCU assembly and promoting efficient assembly of gatekeeper subunits with MCU. Required for paraplegin (SPG7) maturation. After its cleavage by mitochondrial-processing peptidase (MPP), it converts paraplegin into a proteolytically active mature form. Required for the maturation of PINK1 into its 52kDa mature form after its cleavage by mitochondrial-processing peptidase (MPP). Involved in the regulation of OMA1-dependent processing of OPA1.
  • 基因功能參考文獻：
    1. Deletion of AFG3L2 associated with spinocerebellar ataxia type 28 in the context of multiple genomic anomalies. PMID: 25251419
    2. AOA2 with myoclonus associated with mutations in SETX and AFG3L2 PMID: 25927548
    3. This study that AFG3L2 mutations are another important cause, albeit rare, of a late-onset ataxic PEO phenotype due to a disturbance of mtDNA maintenance. PMID: 25420100
    4. StAR proteolysis is executed by at least 2 mitochondrial proteases, the matrix LON protease and the inner membrane complexes of the metalloproteases AFG3L2 and AFG3L2:SPG7/paraplegin. PMID: 24422629
    5. Here, we report on a novel AFG3L2 mutation in a patient with slowly progressive ataxia and a positive family history. PMID: 24293060
    6. Identification of a partial AFG3L2 deletion and subsequent functional studies reveal loss of function as the most likely disease mechanism. PMID: 24814845
    7. Both full-length and truncated COX1 proteins physically interact with AFG3L2. PMID: 22252130
    8. These findings expand the phenotype associated with AFG3L2 mutations and suggest that AFG3L2-related disease should be considered in the differential diagnosis of spastic ataxias. PMID: 22022284
    9. The mutations of SCA28 are associated with amino acid changes in evolutionarily conserved residues of the alleged SCA28 gene, and indicate SCA28 as the sixth recognized SCA genotype caused by point mutations. PMID: 21827917
    10. We further confirm both the involvement of AFG3L2 gene in Spinocerebellar ataxia type 28 (SCA28) and the presence of a mutational hotspot in exons 15-16. PMID: 20725928
    11. in spinocerebellar ataxia type 28 patients study found novel missense mutation at an evolutionarily conserved amino-acid position; amino-acid exchange p.E700K was detected in a 4-generation family and was not observed in chromosomes of controls PMID: 20354562
    12. work identifies AFG3L2 as a novel cause of dominant neurodegenerative disease and indicates a previously unknown role for this component of the mitochondrial protein quality control machinery in protecting the human cerebellum against neurodegeneration. PMID: 20208537
    13. An intersubunit signaling network coordinates ATP hydrolysis by m-AAA protease AGG3L2. PMID: 19748354

    顯示更多

    收起更多

  • 相關疾病：
    Spinocerebellar ataxia 28 (SCA28); Spastic ataxia 5, autosomal recessive (SPAX5)
  • 亞細胞定位：
    Mitochondrion. Mitochondrion inner membrane; Multi-pass membrane protein.
  • 蛋白家族：
    AAA ATPase family; Peptidase M41 family
  • 組織特異性：
    Ubiquitous. Highly expressed in the cerebellar Purkinje cells.
  • 數據庫鏈接：

    HGNC: 315

    OMIM: 604581

    KEGG: hsa:10939

    STRING: 9606.ENSP00000269143

    UniGene: Hs.726355



主站蜘蛛池模板： 久久亚洲国产精品成人av秋霞| 欧美性欧美巨大黑白大战| 国产又黄又刺激又高潮的网站| 人妻夜夜爽天天爽| 伊人久久大香线蕉午夜av| 2020精品国产视| 日本乱人伦aⅴ精品| 欧美人与动牲交免费观看| 亚洲国产精品综合久久20| 午夜不卡久久精品无码免费| 国产高清av在线播放| 污污又黄又爽免费的网站| 国偷自产一区二区三区在线视频| 成人免费无码大片a毛片18| 中文无码精品一区二区三区| 6080yyy午夜理论片中无码 | 亚洲成a人片77777国产| 粗大的内捧猛烈进出看视频| 国产在线一区二区三区四区五区| 精品无人区无码乱码毛片国产| 欧美国产成人精品一区二区三区| 久久人人做人人妻人人玩精品hd| 亚洲一区二区三区波多野结衣| 久久亚洲日韩精品一区二区三区| 国产精品久久久久久久久久免费看| 亚洲精品国产精品国自产| 国产免费丝袜调教视频| 四虎影视884a精品国产| 中文字幕无码av免费久久| 人人妻人人澡人人爽秒播| 久久精品中文騷妇女内射| 亚洲成色综合网站在线| 亚洲人成电影综合网站色www| 久久久精品人妻一区二区三区四| 免费午夜爽爽爽www视频十八禁| 国产欧美二区综合| 国内精品久久久久影院优| 久久国产免费直播| 亚洲欧美日韩国产精品一区二区 | 国产精品第一国产精品| 欧美亚洲日韩国产综合电影|