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

AIPL1 Antibody

  • 中文名稱:
    AIPL1兔多克隆抗體
  • 貨號:
    CSB-PA001501GA01HU
  • 規格:
    ¥3,900
  • 其他:

產品詳情

  • Uniprot No.:
  • 基因名:
    AIPL1
  • 別名:
    A930007I01Rik antibody; Aipl1 antibody; AIPL1_HUMAN antibody; AIPL2 antibody; Aryl hydrocarbon interacting protein like 1 antibody; Aryl hydrocarbon receptor interacting protein like 1 antibody; Aryl-hydrocarbon-interacting protein-like 1 antibody; LCA4 antibody; MGC25485 antibody; OTTHUMP00000128207 antibody; OTTMUSP00000006382 antibody; RP23-401C17.1 antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human,Mouse,Rat
  • 免疫原:
    Human AIPL1
  • 免疫原種屬:
    Homo sapiens (Human)
  • 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, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 產品提供形式:
    Liquid
  • 應用范圍:
    ELISA,WB,IHC
  • 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:
    May be important in protein trafficking and/or protein folding and stabilization.
  • 基因功能參考文獻:
    1. This review summarizes important recent advances in understanding the mechanisms underlying normal function of AIPL1 and the protein perturbations caused by pathogenic mutations PMID: 28939106
    2. findings offer critical insights into the mechanisms that underlie AIPL1 function in health and disease, and highlight the structural and functional diversity of the FKBPs PMID: 28739921
    3. Authors characterized the functional deficits of AIPL1 variations, some of which induce aberrant pre-mRNA AIPL1 splicing leading to the production of alternative AIPL1 isoforms and investigated the ability of the AIPL1 variants to mediate an interaction with HSP90 and modulate the rod cGMP PDE6 stability and activity. PMID: 28973376
    4. Findings suggest that AIPL1 function in retinal photoreceptor cells is not related to the role of EB proteins in microtubule dynamics or primary ciliogenesis, but their association may be related to a specific role in the retinal photoreceptors. PMID: 25799540
    5. The authors established a transgenic mouse model for cone-rod dystrophy carrying human AIPL1 gene with deletion in the C-terminal proline-rich region. PMID: 25274777
    6. Gene therapy based approach may be worthy of consideration in a small group of selected patients with preserved outer retinal structure in AIPL1 Leber's congenital amaurosis. PMID: 25596619
    7. Mutations in the AIPL1 and RDH12 genes associated with leber congenital amaurosis in two Turkish families. PMID: 25148430
    8. In this chapter, using results obtained from multiple lines of animal models, we discuss the role for AIPL1 in photoreceptors. PMID: 24664679
    9. The unique proline-rich domain of AIPL1 is important for its chaperone function as its truncation severely affects the ability of AIPL1 to bind non-native proteins. PMID: 23418749
    10. Patients with mutations in AIPL1 may present with Leber congenital amaurosis and residual ERGs (electroretinography) characterized by slow insensitive scotopic responses. PMID: 21900377
    11. AIPL1-Leber congenital amaurosis (LCA), unlike some other forms of LCA with equally severe visual disturbance, shows profound loss of foveal as well as extrafoveal photoreceptors. PMID: 20702822
    12. AIPL1 is needed for the proper functioning and survival of cone photoreceptors. PMID: 20042464
    13. Variations of macular microstructures were observed among LCA (Leber congenital amaurosis) patients with different genotypes. PMID: 19959640
    14. Phenotype-genotype correlations of AIPL1-associated Leber's congenital amaurosis (LCA) PMID: 11548141
    15. AIPL1 performs a function essential to the maintenance of rod photoreceptor function PMID: 11929855
    16. The inherited blindness associated protein AIPL1 interacts with the cell cycle regulator protein NUB1 in the retina. PMID: 12374762
    17. Interacts with and aids in processing of farnesylated proteins in the retina. PMID: 14555765
    18. interaction between NUB1 and AIPL1 is affected in patients with Leber congenital amaurosis PMID: 15081406
    19. Plays role in cytosolic stability and/or nuclear transport of NUB1 during regulation of cell cyle during photoreceptor development. PMID: 15180275
    20. The phenotype of LCA (Leber congenital amaurosis) in patients with AIPL1 mutations is relatively severe, with a maculopathy in most patients and keratoconus and cataract in a large subset. PMID: 15249368
    21. Data show that aryl hydrocarbon receptor-interacting protein-like 1 (AIPL1) can modulate protein translocation and act in a chaperone-like manner and suggest that AIPL1 is an important modulator of NEDD8 ultimate buster protein 1 (NUB1) cellular function. PMID: 15347646
    22. aryl hydrocarbon interacting protein-like 1 (AIPL1) mutations may have a role in inherited retinal dystrophies PMID: 15469903
    23. AIPL1 may cooperate with both Hsp70 and Hsp90 within retina-specific chaperone heterocomplex, and specialized role of AIPL1 in photoreceptors may therefore be facilitated by these molecular chaperones. PMID: 18408180

    顯示更多

    收起更多

  • 相關疾病:
    Leber congenital amaurosis 4 (LCA4)
  • 亞細胞定位:
    Cytoplasm. Nucleus.
  • 組織特異性:
    Highly expressed in retina. Specifically localized to the developing photoreceptor layer and within the photoreceptors of the adult retina.
  • 數據庫鏈接:

    HGNC: 359

    OMIM: 604392

    KEGG: hsa:23746

    STRING: 9606.ENSP00000370521

    UniGene: Hs.279887



主站蜘蛛池模板: 国产成人夜色高潮福利影视| 亚洲色自偷自拍另类小说| 欧美xxxx做受欧美1314| 色惰日本视频网站www| 国产午夜永久福利视频在线观看| 中国产xxxxa片在线观看| 亚洲人午夜射精精品日韩| 欧美人与动人物姣配xxxx| 日韩欧美国产aⅴ另类| 沈阳45老熟女高潮喷水亮点| 日韩欧美成人免费观看| 人妻综合专区第一页| 熟妇的奶头又大又长奶水视频| 人妻av中文系列| 日韩人妻无码一区二区三区久久99| 国产精自产拍在线看中文| 日本熟妇厨房bbw| 亚洲精品久久一区二区三区777| 一本久道久久综合狠狠爱| 欧美三級片黃色三級片黃色| 亚洲成av人片天堂网| 人妻熟妇乱又伦精品视频中文字幕| 亚洲国产精品无码久久一线| 国产午夜福利精品一区| 亚洲精品一区二区三区中文字幕| 国产97色在线 | 免| 国产精品国语对白露脸在线播放| 国产下药迷倒白嫩美女网站| 精品欧美一区二区在线观看 | 国产69精品久久久久777| 国产在线高清亚洲精品二区| 婷婷丁香五月缴情视频| 色综合中文综合网| 国产免费mv大全视频网站| 国产av国片精品有毛| 51久久国产露脸精品国产| 内射人妻无套中出无码| av网站的免费观看| 男女无遮挡激情视频| 尤物yw193无码点击进入| 色一乱一伦一图一区二区精品|