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: 国产偷久久久精品专区| 国产精品一区二区在线观看| 亚洲精品人成网线在线播放va| 国产成人福利美女观看视频| 国产午夜福利不卡在线观看| 久久www免费人成_看片中文| 成人国产一区二区三区| 97夜夜澡人人爽人人喊a| 窝窝午夜看片成人精品| 国产免费二卡3卡四卡| 人人妻人人澡人人爽欧美一区双| 国产又粗又猛又大爽老大爷| 国产爆乳无码av在线播放| 日韩成人av无码一区二区三区| 再深点灬舒服灬太大了网站 | 亚洲精品色情app在线下载观看| 亚洲精品无码一区二区| 日本一区二区三区免费视频| 国产精品亚洲专区无码老司国| 亚洲日韩中文字幕在线播放| 国产麻豆精品一区二区三区v视界| 精品熟女少妇a∨免费久久| 国产美女露脸口爆吞精| 免费人成视频19674不收费 | 久久亚洲国产最新网站之一| 亚洲一卡2卡3卡4卡5卡乱码观看| 亚洲男人的天堂在线播放| 国产女人与公拘交在线播放| 在线看片免费人成视频国产片 | 粉嫩大学生无套内射无码卡视频| 国产精品爽爽v在线观看无码| 野花社区在线www日本| 国产成人精品999视频| 久久久久人妻一区精品性色av | 无套内谢孕妇毛片免费看| 国产美女无遮挡裸色视频| 成人女人黄网站免费视频| 日本高清色倩视频在线观看| 最爽无遮挡行房视频| 日韩成人无码v清免费| 免费毛儿一区二区十八岁|