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

CRYBB1 Antibody

  • 中文名稱:
    CRYBB1兔多克隆抗體
  • 貨號:
    CSB-PA006012ESR2HU
  • 規格:
    ¥440
  • 圖片:
    • Western blot
      All lanes: CRYBB1 antibody at 1.58µg/ml
      Lane 1: MCF-7 whole cell lysate
      Lane 2: Mouse kidney tissue
      Secondary
      Goat polyclonal to Rabbit IgG at 1/10000 dilution
      Predicted band size: 28 kDa
      Observed band size: 28 kDa
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) CRYBB1 Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    CRYBB1
  • 別名:
    Beta crystallin B1 antibody; Beta-B1 crystallin antibody; Beta-crystallin B1 antibody; CATCN3 antibody; CRBB1_HUMAN antibody; CRYBB 1 antibody; Crybb1 antibody; Crystallin beta B1 antibody; CTRCT17 antibody; Eye lens structural protein antibody; OTTHUMP00000028719 antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human, Mouse
  • 免疫原:
    Recombinant Human Beta-crystallin B1 protein (1-252AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標記方式:
    Non-conjugated
  • 克隆類型:
    Polyclonal
  • 抗體亞型:
    IgG
  • 純化方式:
    Antigen Affinity Purified
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
  • 產品提供形式:
    Liquid
  • 應用范圍:
    ELISA, WB
  • 推薦稀釋比:
    Application Recommended Dilution
    WB 1:1000-1:5000
  • 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:
    Crystallins are the dominant structural components of the vertebrate eye lens.
  • 基因功能參考文獻:
    1. We examined a cohort of Chinese patients with congenital cataracts and studied the phenotypes and genotypes. Extralenticular abnormalities, such as microcornea and ocular coloboma, can also be found in patients with congenital cataracts. The phenotype of congenital cataracts associated with macular and optic disc coloboma was reported for the first time in this study. PMID: 29386872
    2. Our findings highlight the importance of the C-terminus in betaB1-crystallin in maintaining the crystalline function and stability, and provide a novel insight into the molecular mechanism underlying the pathogenesis of human autosomal dominant congenital cataract. PMID: 28928627
    3. CRYBB1 partial duplication ad complete duplication of CRYBA4 identified in a family with autosomal dominant congenital cataract. PMID: 28272538
    4. Molecular dynamic simulation studies indicated that the mutation decreased the subunit binding energy and modified the distribution of surface electrostatic potentials. More importantly, the mutation separated two interacting loops in the C-terminal domain, which shielded the hydrophobic core from solvent in native betaB1-crystallin. PMID: 27318838
    5. Congenital microcornea-cataract syndrome-causing mutation X253R increases betaB1-crystallin hydrophobicity to promote aggregate formation PMID: 27208166
    6. Despite the disruption of betaB1-crystallin assembly, the thermal stability of betaB1-crystallin was increased by the mutation accompanied by the reduction of thermal aggregation at high temperatures PMID: 23159606
    7. Sequencing of this gene revealed a homozygous c.171del mutation (p.N58Tfs*107) with a shared haplotype in all 16 children. PMID: 22267527
    8. study identified a novel heterozygous p.Ser129Arg mutation in CRYBB1 in a congenital cataract-microcornea syndrome family of Chinese origin PMID: 21972112
    9. The formation of beta-crystallin heteromers not only stabilizes the unstable acidic beta-crystallin but also protects them against aggregation during refolding from the stress-denatured states. PMID: 22032798
    10. The presence of significant amounts of small peptides derived from gammaS- and betaB1-crystallins in the water-insoluble fraction of the lens indicates that these interact tightly with cytoskeletal or membrane components. PMID: 21447408
    11. Analyses of 20 Chinese families with hereditary nuclear congenital cataract revealed 3 novel mutations. Two of these mutations (V146M and I21N) affected betaB2-crystallin (CRYBB2). One mutation (R233H) was detected in betaB1-crystallin (CRYBB1). PMID: 21402992
    12. Variant alleles of the CRYBB1 and CRYBB2 genes were found, none are considered pathogenic. PMID: 20565250
    13. Data show a significant demixing of gammaD and betaB1 i.e., large difference of composition in the two coexisting phases. PMID: 20616077
    14. Mutation G220X is associated with autosomal dominant cataract. PMID: 12360425
    15. 1.4 angstroms resolution crystal structure of a truncated version of human betaB1 that resembles an in vivo age-related truncation PMID: 14573871
    16. The molecular characterization of a UK family with an autosomal dominant congenital cataract associated with microcornea is reported PMID: 16110300
    17. fundamental transcriptional regulatory mechanism of the betaB1-crystallin gene has been well conserved between humans and zebrafish PMID: 16331646
    18. the sequence of betaB2-crystallin appears well optimized for domain swapping PMID: 17327390
    19. The dimeric intermediate may be a critical determinant for the life-long stability of the beta-crystallins and has important consequences on interactions with alpha-crystallin. PMID: 17448466
    20. The current study showed that a different mutation in the same gene causes an autosomal recessive form of the disease. PMID: 17460281
    21. study identified a novel mutation in CRYBB1 gene in a Chinese family with autosomal dominant congenital cataract; results provide strong evidence that CRYBB1 is a pathogenic gene for congenital cataract PMID: 17531125
    22. This study has identified a novel nonsense mutation in CRYBB1 (p.Q223X) associated with autosomal dominant congenital nuclear cataract. PMID: 18432316
    23. study identified an initiation codon mutation in CRYBB1 in a family with autosomal recessive form of congenital cataract (nuclear pulverulent cataract) PMID: 19461930

    顯示更多

    收起更多

  • 相關疾病:
    Cataract 17, multiple types (CTRCT17)
  • 蛋白家族:
    Beta/gamma-crystallin family
  • 數據庫鏈接:

    HGNC: 2397

    OMIM: 600929

    KEGG: hsa:1414

    STRING: 9606.ENSP00000215939

    UniGene: Hs.37135



主站蜘蛛池模板: 久久国产超碰女女av| 亚洲国产精品无码久久青草| 蜜桃日本免费观看mv| 久久精品亚洲综合专区| 无码成a毛片免费| 无码高潮少妇毛多水多水免费| 一本一道av中文字幕无码| 欧美亚洲另类自拍丝袜| 免费无码毛片一区二区三区a片 | 国产精品内射视频免费| 玩弄人妻少妇老师美妇厨房| 亚洲精品日本久久一区二区三区| 国产极品美女高潮抽搐免费网站| 黑人巨大videos精品| 精品人妻潮喷久久久又裸又黄| 免费看成人aa片无码视频吃奶| 老牛精品亚洲成av人片| 亚洲欧美成人综合图区| 日本少妇自慰免费完整版| 国产女主播喷水视频在线观看| 亚洲偷自拍国综合| 丰满的少妇邻居中文bd| 麻豆国产一区二区三区四区| 国产精品毛片无码| 中文字幕无码日韩专区免费| 久久99国产综合精品女同| 成人一区二区免费视频| 色偷偷88888欧美精品久久久| 午夜男女无遮挡拍拍视频| 大香网伊人久久综合网2018 | 亚洲成a∧人片在线观看无码| 国产传媒精品1区2区3区| 亚洲欧美日韩精品久久| 在线日韩日本国产亚洲| 国产亚洲va在线电影| 浪潮av激情高潮国产精品| 精品夜夜澡人妻无码av| 欧美婷婷六月丁香综合| 无尺码精品产品网站| 免费观看18禁无遮挡真人网站| 成年片色大黄全免费网站久久|