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SEC23B Antibody

  • 货号：
    CSB-PA621874LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • IHC image of CSB-PA621874LA01HU diluted at 1:300 and staining in paraffin-embedded human small intestine tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
    • IHC image of CSB-PA621874LA01HU diluted at 1:300 and staining in paraffin-embedded human appendix tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) SEC23B Polyclonal antibody
  • Uniprot No.：
    Q15437
  • 基因名：
  • 别名：
    CDA II antibody; CDAII antibody; CDAN2 antibody; HEMPAS antibody; Protein transport protein Sec23B antibody; RP11-379J5.1 antibody; SC23B_HUMAN antibody; Sec23 homolog B (S. cerevisiae) antibody; SEC23 related protein B antibody; SEC23-like protein B antibody; SEC23-related protein B antibody; Sec23b antibody; Transport protein SEC23B antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Protein transport protein Sec23B protein (208-325AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，SEC23B Antibody (CSB-PA621874LA01HU)，的标记方式是Non-conjugated。对于SEC23B Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA621874LB01HU SEC23B Antibody, HRP conjugated ELISA
    FITC CSB-PA621874LC01HU SEC23B Antibody, FITC conjugated
    Biotin CSB-PA621874LD01HU SEC23B Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, IHC
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:200-1:500
  • 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：
    Component of the coat protein complex II (COPII) which promotes the formation of transport vesicles from the endoplasmic reticulum (ER). The coat has two main functions, the physical deformation of the endoplasmic reticulum membrane into vesicles and the selection of cargo molecules for their transport to the Golgi complex.
  • 基因功能参考文献：
    1. A Y462C mutation was found in 5 members of a consanguineous Indian family. In the homozygous patient, it resulted in congenital dyserythropoietic anemia type II. PMID: 28879554
    2. described the functional interaction between GATA1 and SEC23B genes in two patients with suspected congenital dyserythropoietic anemia type II PMID: 28550189
    3. these data demonstrate an equivalent function for SEC23A/B, with evolutionary shifts in the transcription program likely accounting for the distinct phenotypes of SEC23A/B deficiency within and across species, a paradigm potentially applicable to other sets of paralogous genes. PMID: 30065114
    4. novel compound mutations of c.1727T>C and c.1831C>T of the SEC23B gene probably underlie the congenital dyserythropoietic anemia type II in the family PMID: 29188620
    5. Mutation in SEC23B gene is associated with congenital dyserythropoietic anemia. PMID: 29031773
    6. miR-130a is an epigenetically regulated miRNA involved in regulation of key molecular and phenotypic features of prostate carcinogenesis, acting as a tumour suppressor miRNA by targeting SEC23B and DEPDC1. PMID: 27984115
    7. SEC23B-Y462C congenital dyserythropoietic anemia type II is only seen in a distinct Indian community (Vaish) in whom a recessively inherited shared haplotype can be showed, consistent with a founder effect. PMID: 25418799
    8. Germline Heterozygous Variants in SEC23B Are Associated with Cowden Syndrome and Enriched in Apparently Sporadic Thyroid Cancer. PMID: 26522472
    9. Congenital dyserythropoietic anemia, type II with SEC23B exon 12 c.1385 A --> G mutation, and pseudo-Gaucher cells in two siblings has been described. PMID: 24801240
    10. Both probands with congenital dyserythropoietic anemia IotaI in the second family were homozygotes of the SEC23B gene with mutation c.938G>A (R313H). PMID: 24196372
    11. Mutations in SEC23B lead to congenital dyserythropoietic anemia type II due to alterations in coat protein (COP)II complex trafficking machinery. [REVIEW] PMID: 22764119
    12. study identified four novel SEC23B mutations associated with ongenital dyserythropoietic anemia type II disease; also demonstrated that the genetic alteration results in a significant decrease of SEC23B transcript in erythroid precursors PMID: 22208203
    13. Data indicate that SEC23B founder mutations E109K and R14W account for about 54% of all mutations in CDA II patients in Italy; data suggest R14W occurred Southern Italy, E109K is more widespread within Europe. PMID: 21850656
    14. Correlation between SEC23B mutations and congenital dyserythropoietic anemia type II parameters shows that addition of one missense mutation and one nonsense mutation tends to produce a more severe presentation then association of two missense mutations. PMID: 20015893
    15. Most congenital dyserythropoietic anemia II patients in Israel are of Moroccan Jewish origin and carry a common SEC23B mutation, E109K, the first to be described as a founder mutation causing CDA II. PMID: 21252497
    16. found 19 novel variants in the homozygous or the compound heterozygous state in 28 CDA II patients from 21 unrelated families enrolled in the CDA II International Registry PMID: 20941788
    17. This study found SEC23B mutations in two patients previously classified as atypical congenital dyserythropoietic anemias presenting with hydrops foetalis. PMID: 20381388
    18. Hetero- or homozygous mutation of CDAN2 causes hypoglycosylation of band 3, accumulation and hypoglycosylation of polyglycosylceramides, and accumulation of lactotriaosylceramide. PMID: 11836161
    19. These results provide in vivo evidence for SEC23B selectivity in erythroid differentiation and show that SEC23A and SEC23B, although highly related paralogous secretory COPII components, are nonredundant in erythrocyte maturation. PMID: 19561605
    20. Congenital dyserythropoietic anemia type II (CDAII) is caused by mutations in the SEC23B gene. PMID: 19621418

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  • 相关疾病：
    Cowden syndrome 7 (CWS7); Anemia, congenital dyserythropoietic, 2 (CDAN2)
  • 亚细胞定位：
    Cytoplasmic vesicle, COPII-coated vesicle membrane; Peripheral membrane protein; Cytoplasmic side. Endoplasmic reticulum membrane; Peripheral membrane protein; Cytoplasmic side. Cytoplasm, cytosol.
  • 蛋白家族：
    SEC23/SEC24 family, SEC23 subfamily
  • 组织特异性：
    Ubiquitously expressed.
  • 数据库链接：

    HGNC: 10702

    OMIM: 224100

    KEGG: hsa:10483

    STRING: 9606.ENSP00000262544

    UniGene: Hs.369373