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

MFSD8 Antibody, HRP conjugated

  • 中文名稱:
    MFSD8兔多克隆抗體, HRP偶聯
  • 貨號:
    CSB-PA844087LB01HU
  • 規格:
    ¥880
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) MFSD8 Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    MFSD8
  • 別名:
    Ceroid-lipofuscinosis neuronal protein 7 antibody; CLN7 antibody; Major facilitator superfamily domain-containing protein 8 antibody; MFSD8 antibody; MFSD8_HUMAN antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human
  • 免疫原:
    Recombinant Human Major facilitator superfamily domain-containing protein 8 protein (1-40AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標記方式:
    HRP
  • 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:
    May be a carrier that transport small solutes by using chemiosmotic ion gradients.
  • 基因功能參考文獻:
    1. This study highlights a hierarchy of MFSD8 variant severity, predicting three consequences of mutation: (1) nonsyndromic localized maculopathy, (2) nonsyndromic widespread retinopathy, or (3) syndromic neurological disease. PMID: 28586915
    2. MFSD8 genetic testing should also be considered in patients with Rett like phenotype at onset and negative MECP2 mutation PMID: 25439737
    3. A mutation in MFSD8, c.472G>A (p.Gly158Ser), segregates with the disease phenotype in variant late infantile neuronal ceroid lipofuscinosis. PMID: 25270050
    4. In this study, we identified variants in MFSD8 as a novel cause of nonsyndromic autosomal recessive macular dystrophy with central cone involvement. PMID: 25227500
    5. This study showed that Gene disruption of Mfsd8 provides animal model for CLN7 disease. PMID: 24423645
    6. Expression and lysosomal targeting of CLN7 are reported. PMID: 20826447
    7. MFSD8 gene is involved in late-infantile-onset neuronal ceroid lipofuscinose;it was mapped to chromosome 4q28.1-q28.2. PMID: 17564970
    8. Results describe a novel mutation in the MFSD8 gene, responsible for neuronal ceroid lipofuscinoses, in a consanguineous Egyptian family PMID: 18850119
    9. Study contributes to a better molecular characterization of Italian NCL cases, and will facilitate medical genetic counseling in such families. PMID: 19177532
    10. CLN7/MFSD8 defects are not restricted to the Turkish population, as initially anticipated, but are a relatively common cause of NCL in different populations. PMID: 19201763
    11. Data show that neuronal ceroid lipofuscinosis in a Saudi family is due to a homozygous novel mutation in the most recently described NCL gene (MFSD8). PMID: 19277732

    顯示更多

    收起更多

  • 相關疾病:
    Ceroid lipofuscinosis, neuronal, 7 (CLN7); Macular dystrophy with central cone involvement (CCMD)
  • 亞細胞定位:
    Lysosome membrane; Multi-pass membrane protein. Note=Sorting to lysosomes involves tyrosine- and/or dileucine-based motifs.
  • 蛋白家族:
    Major facilitator superfamily
  • 組織特異性:
    Expressed at very low levels in all tissues tested.
  • 數據庫鏈接:

    HGNC: 28486

    OMIM: 610951

    KEGG: hsa:256471

    STRING: 9606.ENSP00000296468

    UniGene: Hs.480701



主站蜘蛛池模板: 国产极品精品自在线| 国产特黄级aaaaa片免| 无码av免费精品一区二区三区 | 无码国模大尺度视频在线观看 | 欧美金妇欧美乱妇xxxx| 午夜性色福利在线观看视频| 精品亚洲成av人在线观看| 亚洲精品久久久狠狠爱小说| 精品免费久久久久久久| 国产aⅴ视频免费观看| 国产sp调教打屁股视频网站| 香蕉久久精品日日躁夜夜躁夏 | 日韩精品中文字幕无码一区| 欧美人禽杂交狂配免费看| 嫩草欧美曰韩国产大片| 欧美日韩色另类综合| 夜夜躁狠狠躁日日躁孕妇| av片亚洲国产男人的天堂| 国模杨依粉嫩蝴蝶150p| 国产最新进精品视频| 中文精品一卡2卡3卡4卡国色| 亚洲一本一道一区二区三区| 99久久亚洲精品无码毛片 | 2020国产成人精品影视| 国产成人精品三级麻豆| 51妺嘿嘿午夜福利| av无码中文字幕不卡一区二区三区| 亚洲人成人无码www影院 | 少妇被黑人到高潮喷出白浆| 久久精品国产久精国产果冻传媒| 在线观看片免费视频无码| 国精产品一二三区精华液| 偷偷要色偷偷中文无码| 亚洲人成小说网站色在线观看| 亚洲中文字幕无码不卡电影| 亚洲精品久久国产高清情趣图文| 中文字幕精品视频在线看免费 | 老男人久久青草av高清| 天美麻花果冻视频大全英文版| 欧美一区二区三区视频在线观看 | 亚洲人成在线观看网站不卡 |