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AP3B1 Antibody

  • 货号：
    CSB-PA001874ESR1HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Immunohistochemistry of paraffin-embedded human colon cancer using CSB-PA001874ESR1HU at dilution of 1:100
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) AP3B1 Polyclonal antibody
  • Uniprot No.：
    O00203
  • 基因名：
  • 别名：
    Adapter related protein complex 3 beta 1 subunit antibody; Adapter-related protein complex 3 subunit beta-1 antibody; Adaptor protein complex AP-3 subunit beta-1 antibody; Adaptor protein complex AP3 beta1 subunit antibody; ADTB3 antibody; ADTB3A antibody; AP-3 complex subunit beta-1 antibody; AP3 complex beta1 subunit antibody; AP3B1 antibody; AP3B1_HUMAN antibody; Beta-3A-adaptin antibody; Beta3A adaptin antibody; Clathrin assembly protein complex 3 beta 1 large chain antibody; Clathrin assembly protein complex 3 beta-1 large chain antibody; HPS antibody; PE antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human AP-3 complex subunit beta-1 protein (895-1094AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen Affinity Purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, IHC
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:20-1:200
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    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.

产品评价

靶点详情

  • 功能：
    Subunit of non-clathrin- and clathrin-associated adaptor protein complex 3 (AP-3) that plays a role in protein sorting in the late-Golgi/trans-Golgi network (TGN) and/or endosomes. The AP complexes mediate both the recruitment of clathrin to membranes and the recognition of sorting signals within the cytosolic tails of transmembrane cargo molecules. AP-3 appears to be involved in the sorting of a subset of transmembrane proteins targeted to lysosomes and lysosome-related organelles. In concert with the BLOC-1 complex, AP-3 is required to target cargos into vesicles assembled at cell bodies for delivery into neurites and nerve terminals.
  • 基因功能参考文献：
    1. point mutation c.2702C>G in exon 23 of the AP3B1 gene caused deletion of 112 bp in the mRNA in two siblings. This mutation activates a cryptic donor splice site that overrules the wild-type donor splice site of this exon. Three other novel mutations in AP3B1 were identified, that is, a nonsense mutation c.716G>A (p.Trp239Ter), a 1-bp and a 4-bp deletion c.177delA and c.1839_1842delTAGA, respectively PMID: 28585318
    2. Synergistic defects of UNC13D and AP3B1 leading to adult hemophagocytic lymphohistiocytosis. PMID: 25980904
    3. Using a co-affinity purification strategy, s have identified the beta subunit of the AP-3 adapter protein complex, AP3B1, as a binding partner for the M proteins of the zoonotic paramyxoviruses Nipah virus and Hendra virus. PMID: 25210190
    4. Data indicate that RUN and FYVE domain protein Rabip4'(RUFY1) interacts specifically and directly with adaptor protein complex AP-3. PMID: 23144738
    5. The study provides a description of two unrelated individuals with Hermansky Pudlak syndrome type 2 associated with novel mutations in AP3B1. PMID: 19679886
    6. diphosphoinositol pentakisphosphate-mediated pyrophosphorylation of AP3B1 modulates the interaction with Kif3A and, as a consequence, affects the release of HIV-1 virus-like particles. PMID: 19934039
    7. Two nonsense mutations in ADTB3A, C1578T (R-->X) and G2028T (E-->X), result in lack of ADTB3A mRNA and beta3A protein production and a severe, G-CSF-responsive neutropenia in addition to oculocutaneous albinism and platelet storage pool deficiency. PMID: 11809908
    8. Description of mutations in HPS genes that cause Hermansky-Pudlak syndrome (review) PMID: 12125811
    9. We defined a homozygous genomic deletion in AP3B1, the gene encoding the beta chain of the adaptor protein-3 (AP-3) complex. The mutation leads to in-frame skipping of exon 15 and thus perturbs proper assembly of the heterotetrameric AP-3 complex. PMID: 16537806
    10. A novel homozygous mutation in AP3B1 was detected in a 2-year-old patient with oculocutaneous albinism and immunodeficiency with Hermansky-Pudlak syndrome type II and eventual acute fatal hemophagocytic lymphohistiocytosis. PMID: 16551969
    11. Mutations in AP3B1 is not associated with familial hemophagocytic lymphohistiocytosis PMID: 18000860
    12. AP-1 and AP-3 are involved in the formation of distinct types of clathrin-coated vesicles, each of which is characterized by the incorporation of specific cargo membrane proteins PMID: 18410487
    13. Experimental investigation of five specific genes, AP3B1, ATP6AP1, BLOC1S1, LAMP2, and RAB11A, has confirmed novel roles for these proteins in the proper initiation of macroautophagy in amino acid-starved fibroblasts. PMID: 19246570

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  • 相关疾病：
    Hermansky-Pudlak syndrome 2 (HPS2)
  • 亚细胞定位：
    Cytoplasmic vesicle, clathrin-coated vesicle membrane; Peripheral membrane protein; Cytoplasmic side. Golgi apparatus.
  • 蛋白家族：
    Adaptor complexes large subunit family
  • 组织特异性：
    Ubiquitously expressed.
  • 数据库链接：

    HGNC: 566

    OMIM: 203300

    KEGG: hsa:8546

    STRING: 9606.ENSP00000255194

    UniGene: Hs.532091