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

IFT140 Antibody, FITC conjugated

  • 中文名稱:
    IFT140兔多克隆抗體, FITC偶聯
  • 貨號:
    CSB-PA842756LC01HU
  • 規格:
    ¥880
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) IFT140 Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    IFT140
  • 別名:
    IFT140 antibody; KIAA0590 antibody; WDTC2 antibody; Intraflagellar transport protein 140 homolog antibody; WD and tetratricopeptide repeats protein 2 antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human
  • 免疫原:
    Recombinant Human Intraflagellar transport protein 140 homolog protein (1291-1462AA)
  • 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:
    Component of the IFT complex A (IFT-A), a complex required for retrograde ciliary transport and entry into cilia of G protein-coupled receptors (GPCRs). Plays a pivotal role in proper development and function of ciliated cells through its role in ciliogenesis and/or cilium maintenance. Required for the development and maintenance of the outer segments of rod and cone photoreceptor cells. Plays a role in maintenance and the delivery of opsin to the outer segment of photoreceptor cells.
  • 基因功能參考文獻:
    1. A maternally inherited homozygous biallelic mutation altering the exon 6 splice donor site in IFT140 gene causes Mainzer-Saldino syndrome. PMID: 28724397
    2. We provide the first description of an Opitz trigonocephaly C syndrome (OTCS) phenotype that appears to result from IFT140 mutations. The presentation of this patient is consistent with previous reports showing that OTCS already exhibited skeleletal and nonskeletal features of a ciliopathy PMID: 27874174
    3. Recessive IFT140 mutations cause a severe congenital retinal dystrophy with high hyperopia and often early photophilia. Developmental delay is common but not universal and not all patients have obvious extraocular findings. PMID: 26359340
    4. This study highlights the phenotype of nonsyndromic RP due to mutations in IFT140 with milder retinal dystrophy than that associated with the syndromic disease. PMID: 26968735
    5. Identification of IFT140 variants in multiple unrelated non-syndromic Leber congenital amaurosis and retinitis pigmentosa. PMID: 26216056
    6. Genetic analysis revealed both to harbor recessive mutations in IFT140, a cilium gene recently associated with the skeletal ciliopathy conorenal syndrome. PMID: 24698627
    7. present study strengthens the rationale for IFT140 screening in skeletal ciliopathy spectrum patients that have kidney disease and/or retinal dystrophy PMID: 23418020
    8. IFT140 mutations were identified in Mainzer-Saldino syndrome. IFT140 plays a role in proper development and function of ciliated cells. PMID: 22503633
    9. loss of Ift140 causes pronounced renal cystic disease and suggest that abnormalities in several different pathways may influence cyst progression. PMID: 22282595

    顯示更多

    收起更多

  • 相關疾病:
    Short-rib thoracic dysplasia 9 with or without polydactyly (SRTD9)
  • 亞細胞定位:
    Cytoplasm, cytoskeleton, cilium basal body. Cytoplasm, cytoskeleton, microtubule organizing center, centrosome. Cell projection, cilium.
  • 數據庫鏈接:

    HGNC: 29077

    OMIM: 266920

    KEGG: hsa:9742

    STRING: 9606.ENSP00000406012

    UniGene: Hs.389438



主站蜘蛛池模板: 国产粉嫩嫩00在线正在播放| 国产精品午夜福利视频234区 | 国产精品无码mv在线观看| 精品国精品国产自在久国产不卡| 国产成人年无码av片在线观看 | 中文字幕精品亚洲无线码vr | 亚洲一卡2卡三卡4卡无卡下载| 亚洲成av人片在线播放无码| 亚洲最大天堂无码精品区| 国产精品亚洲成在人线| 国产成人免费永久播放视频平台| 天天狠天天添日日拍| 强奷乱码中文字幕熟女一| 亚欧无线一线二线三线区别| 日韩卡二卡三卡四卡永久入口| 亚洲丰满熟妇在线播放电影全集| 欧美s码亚洲码精品m码| 天堂а√在线官网| 十八禁午夜福利免费网站| 亚洲精品国偷拍自产在线麻豆| 国产欧美日韩综合精品二区| 中文午夜乱理片无码| 亚洲精品av网站在线观看| 狠狠色噜噜狠狠狠狠7777米奇| 女人和拘做爰正片视频| 亚洲经典国产精华液单品榜| 国产精品夜夜春夜夜爽久久小说| 麻花传媒在线mv免费观看视频 | 国产精品久久久天天影视香蕉| 99re久久精品国产| 在线看片免费人成视频福利| 无码人妻精品一区二区三区久久 | 很色很爽很黄裸乳视频| 国产精品无码久久综合网| 久久久国产精品| 国产激情无码一区二区三区| 欧美人禽杂交狂配免费看| 国产亚洲精品久久久久久老妇小说| 99国产精品国产精品九九| 一二三四视频社区3在线高清| 国产卡一卡二卡三精品|