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: 成人综合网站| 青青草免费公开视频| 亚洲a∨无码无在线观看| 久热国产vs视频在线观看| 亚洲熟妇丰满大屁股熟妇| 99久热在线精品视频观看| 无码人妻丰满熟妇区毛片18| 国产揄拍国产精品| 日韩av无码中文无码电影| 99er热精品视频| xx性欧美肥妇精品久久久久久| 精品夜夜澡人妻无码av| 国产美女裸体丝袜喷水视频| 亚洲欧美国产va在线播放| 深夜福利小视频在线观看| 久久精品国产精品亚洲| 亚洲影院天堂中文av色| 亚洲精品一区二区精华液| 欧美三级a做爰在线观看| a片免费视频在线观看| 中文字字幕在线中文无码| 四虎影视无码永久免费| 国产第一页浮力影院草草| 色老板精品视频在线观看| 天天躁日日躁狠狠躁| 成 人影片 免费观看在线| 国产乱人伦真实精品视频| 西西人体www大胆高清| 女同亚洲一区二区无线码| 人妻aⅴ中文字幕无码| 一本无码久本草在线中文字幕dvd 337p日本欧洲亚洲大胆裸体艺术 国产av一区二区三区 | 伊伊人成亚洲综合人网7777| 中文无码精品a∨在线观看不卡| 男女啪啪抽搐高潮动态图| 999久久久国产精品| 国产精品无码一区二区在线| 久久婷婷人人澡人人爽人人喊| 亚洲 熟女 久久 国产| 精品国产av无码一区二区三区| 久久香综合精品久久伊人| 在线播放偷拍一区精品|