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

PHKA2 Antibody

  • 中文名稱:
    PHKA2兔多克隆抗體
  • 貨號:
    CSB-PA572789
  • 規格:
    ¥1100
  • 圖片:
    • The image on the left is immunohistochemistry of paraffin-embedded Human prostate cancer tissue using CSB-PA572789(PHKA2 Antibody) at dilution 1/30, on the right is treated with synthetic peptide. (Original magnification: ×200)
    • The image on the left is immunohistochemistry of paraffin-embedded Human brain tissue using CSB-PA572789(PHKA2 Antibody) at dilution 1/30, on the right is treated with synthetic peptide. (Original magnification: ×200)
  • 其他:

產品詳情

  • Uniprot No.:
  • 基因名:
  • 別名:
    PHKA2 antibody; PHKLA antibody; PYKPhosphorylase b kinase regulatory subunit alpha antibody; liver isoform antibody; Phosphorylase kinase alpha L subunit antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human
  • 免疫原:
    Synthetic peptide of Human PHKA2
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標記方式:
    Non-conjugated
  • 抗體亞型:
    IgG
  • 純化方式:
    Antigen affinity purification
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
  • 產品提供形式:
    Liquid
  • 應用范圍:
    ELISA,IHC
  • 推薦稀釋比:
    Application Recommended Dilution
    ELISA 1:1000-1:2000
    IHC 1:25-1:100
  • 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:
    Phosphorylase b kinase catalyzes the phosphorylation of serine in certain substrates, including troponin I. The alpha chain may bind calmodulin.
  • 基因功能參考文獻:
    1. Clinical data and analyzed the PHKA2 gene of 17 Chinese male patients suspected of having Glycogen storage disease (GSD) type IXa; study detected 14 mutations in 17 patients, including 8 novel mutations; exons 2 and 4 were hot spots. PMID: 28627441
    2. In this study we summarized the PHKA2 mutation spectrum in Korean glycogen storage disease type IX patients and found that the most common mutation type was gross deletion. PMID: 27103379
    3. The present case also represents the first known reported case of liver PhK deficiency with an PHKA2 mutation and liver cirrhosis. PMID: 21857251
    4. We found that the missense mutation p.Pro1205Leu in the PHKA2 gene is a common cause of hepatic phosphorylase-kinase deficiency in Dutch patients, suggesting a founder-effect PMID: 21911307
    5. two novel mutations found in two GSD type IX patients with different residual enzyme activities PMID: 21131218
    6. Gene analysis was confirmed to represent a useful procedure for diagnosing x-linked liver glycogenosis, for which liver biopsy had previously been required to detect hepatic phosphorylase kinase deficiency PMID: 12862311
    7. Alu-mediated, large deletion-spanning introns 19-26 in PHKA2 is associated with X-linked liver glycogenosis PMID: 17581768
    8. Results show that phosphorylase kinase (PhK) alpha subunit missense mutations or small in-frame deletions/insertions may have a direct impact on the PhK alpha functions. PMID: 18950708

    顯示更多

    收起更多

  • 相關疾病:
    Glycogen storage disease 9A (GSD9A)
  • 亞細胞定位:
    Cell membrane; Lipid-anchor; Cytoplasmic side.
  • 蛋白家族:
    Phosphorylase b kinase regulatory chain family
  • 組織特異性:
    Predominantly expressed in liver and other non-muscle tissues.
  • 數據庫鏈接:

    HGNC: 8926

    OMIM: 300798

    KEGG: hsa:5256

    STRING: 9606.ENSP00000369274

    UniGene: Hs.54941



主站蜘蛛池模板: 亚洲国产精品18久久久久久| 300部国产真实乱| 亚洲另类无码一区二区三区| 野狼第一精品社区| 免费人成在线视频无码| 伊人久久大香线焦av色| 久久99国产综合精品| 国产麻豆剧果冻传媒一区| 久久中文字幕乱码久久午夜| 日韩免费无码成人久久久久久片 | 狠狠色噜噜狠狠狠狠米奇777| 人妻av无码专区| 全部免费毛片在线播放| 欧美不卡一卡二卡三卡| 国产亚洲va在线电影| 欧美日韩一区二区三区在线观看视频| 久久99精品国产麻豆| 播五月开心婷婷欧美综合| 久久综合亚洲欧美成人| a国产一区二区免费入口| 曰韩少妇内射免费播放| 18禁男女污污污午夜网站免费暖暖| 国产激情高中生呻吟视频 | 欧美成人午夜免费全部完| 看黄a大片爽爽影院免费无码 | 亚洲综合久久一本久道| 精品国产18久久久久久| 色伊人亚洲综合网站| av在线播放日韩亚洲欧我不卡| a亚洲va欧美va国产综合| 日日鲁鲁鲁夜夜爽爽狠狠视频97| 老妇女性较大毛片| 精品国产一区二区三区四区在线看 | 久久国产精品国产四虎90后| 好紧好湿好黄的视频| 国产精品沙发午睡系列990531| 国内熟女啪啪自拍| 亚洲欧美国产免费综合视频| 欧美激情在线播放| 久天啪天天久久99久久| 久久婷婷五月综合国产尤物app|