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, FITC conjugated

  • 中文名稱:
    MFSD8兔多克隆抗體, FITC偶聯
  • 貨號:
    CSB-PA844087LC01HU
  • 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:
    FITC
  • 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
  • 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?。?/div>
    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: 亚洲妇女自偷自偷图片| 亚洲精品国产精品无码国模| 亚洲精品宾馆在线精品酒店| 麻豆国产av丝袜白领传媒| 潮喷失禁大喷水aⅴ无码| av香港经典三级级 在线| 亚洲中文字幕无码久久2020| 亚洲超碰无码色中文字幕97| 丰满大肥婆肥奶大屁股 | 久久久久久久久免费看无码| 无码免费毛片手机在线| 久久理论片午夜琪琪电影网| 国产性色αv视频免费| 丰满人妻熟妇乱又伦精品视 | 内射口爆少妇麻豆| 日本无遮挡真人祼交视频| 精品av中文字幕在线毛片 | 国产高清露脸孕妇系列| 久久久久人妻一区二区三区| 四虎影成人精品a片| 麻豆精品一卡二卡三卡| 少妇大胆瓣开下部自慰| 国产成人精品视频ⅴa片软件竹菊 免费精品99久久国产综合精品 | 秋霞午夜一区二区三区黄瓜视频 | 亚洲色欲色欱www在线| 亚欧美日韩香蕉在线播放视频| 国产精品麻豆成人av网| 天码人妻一区二区三区| 成年性生交大片免费看| 国产精品成人免费999| 国产果冻豆传媒麻婆精东| 无码人妻丝袜在线视频| а√8天堂中文官网资源| 免费毛片手机在线播放| 成人av无码国产在线观看 | 久久人人爽爽人人爽人人片av| 国模冰莲极品自慰人体 | 各处沟厕大尺度偷拍女厕嘘嘘| 日韩综合亚洲色在线影院| 免费1级a做爰片在线观看| 免费人成在线观看视频播放|