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TCIRG1 Antibody

  • 货号：
    CSB-PA615690LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • IHC image of CSB-PA615690LA01HU diluted at 1:300 and staining in paraffin-embedded human lung 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-PA615690LA01HU diluted at 1:300 and staining in paraffin-embedded human colon cancer 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.
    • Immunofluorescence staining of HepG2 cells with CSB-PA615690LA01HU at 1:100, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) TCIRG1 Polyclonal antibody
  • Uniprot No.：
    Q13488
  • 基因名：
  • 别名：
    a3 antibody; Atp 6i antibody; Atp6i antibody; ATP6N1C antibody; ATP6V0A3 antibody; ATPase H+ transporting 116kD antibody; OC 116 antibody; OC 116 kDa antibody; OC 116kDa antibody; OC-116 kDa antibody; OC116 antibody; OPTB 1 antibody; OPTB1 antibody; Osteoclastic proton pump 116 kDa subunit antibody; Specific 116 kDa vacuolar proton pump subunit antibody; Stv 1 antibody; Stv1 antibody; T cell immune regulator 1 antibody; T cell immune regulator 1 ATPase H+ transporting lysosomal V0 subunit A antibody; T cell immune regulator 1 ATPase H+ transporting lysosomal V0 subunit A3 antibody; T cell immune response cDNA 7 antibody; T cell immune response cDNA7 protein antibody; T cell, immune regulator 1, ATPase, H+ transporting, lysosomal V0 protein a antibody; T-cell immune regulator 1 antibody; T-cell immune response cDNA7 protein antibody; TCIRG 1 antibody; TCIRG1 antibody; TIRC 7 antibody; TIRC7 antibody; V ATPase 116 kDa antibody; V ATPase 116 kDa isoform a3 antibody; V type proton ATPase 116 kDa subunit a antibody; V-ATPase 116 kDa isoform a3 antibody; V-type proton ATPase 116 kDa subunit a isoform 3 antibody; Vacuolar proton translocating ATPase 116 kDa subunit A antibody; Vacuolar proton translocating ATPase 116 kDa subunit a isoform 3 antibody; Vph 1 antibody; Vph1 antibody; VPP3_HUMAN antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human V-type proton ATPase 116 kDa subunit a isoform 3 protein (76-182AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，TCIRG1 Antibody (CSB-PA615690LA01HU)，的标记方式是Non-conjugated。对于TCIRG1 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-PA615690LB01HU TCIRG1 Antibody, HRP conjugated ELISA
    FITC CSB-PA615690LC01HU TCIRG1 Antibody, FITC conjugated
    Biotin CSB-PA615690LD01HU TCIRG1 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, IF
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:200-1:500
    IF 1:50-1:200
  • 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：
    Part of the proton channel of V-ATPases. Seems to be directly involved in T-cell activation.
  • 基因功能参考文献：
    1. In this study, whole exome sequencing (WES) was successfully used in six patients with malignant infantile osteopetrosis (MIOP) and identified mutations in four MIOP-related genes (CLCN7, TCIRG1, SNX10, and TNFRSF11A). PMID: 27187610
    2. Case Reports: TCIRG1-dependent osteopetrosis with a mild clinical course in Chinese patients. PMID: 28816234
    3. TIRC7 is involved in inflammation in multiple sclerosis and anti-TIRC7 mAb can prevent immune activation via selective inhibition of Th1- and Th17-associated cytokine expression. PMID: 24526664
    4. Since a3 subunit of V-ATPase complex plays a crucial role in bone resorption process, structurally abnormal a3 subunit might have adversely affected bone resorption process, leading to infantile osteopetrosis in Pakistani family. PMID: 29237407
    5. TCIRG1 may be involved in endolysosomal transport-a process known to be important to development of early onset AD. PMID: 28738127
    6. TCIRG1 gene mutation in a Chinese family is associated with infantile malignant osteopetrosis. PMID: 28604959
    7. Nine rare missense variants at evolutionarily conserved sites in TCIRG1 are associated with lower absolute neutrophil count. PMID: 27229898
    8. The highly invasive human breast cancer cell lines express higher levels of the a3 isoform than poorly invasive lines; knockdown of a3 reduces both expression of V-ATPases at the plasma membrane and in vitro invasion of breast tumor cells. (Review) PMID: 26906430
    9. an intronic region in TCIRG1 that seems to be particularly prone to splicing mutations, allowing the production of a small amount of protein sufficient to reduce the severity of the phenotype usually associated with TCIRG1 defects. PMID: 25829125
    10. TIRC7 might be involved in the pathogenesis of aplastic anemia. PMID: 26049920
    11. An A to T transversion in the fourth base of the intron 2 donor splice site (c.117+4A-->T) in TCIRG1 in the Ashkenazi Jewish (AJ) population was found to be responsible for osteopetrosis. PMID: 24989235
    12. TIRC7 might be associated with the pathogenesis of ITP, and TIRC7 levels could be used as an indicator to evaluate patients' response to HD-DXM treatment. PMID: 24617318
    13. Increased expression of TIRC7 in plasma was associated with the severity of acute graft-versus-host disease. PMID: 25623380
    14. Data indicate that the effects of epiregulin (EREG) and V-ATPase (TCIRG1) single nucleotide polymorphism (SNP) on pulmonary tuberculosis susceptibility, to the extent that they exist, are dependent on gene-gene interactions in West African populations. PMID: 24898387
    15. TCIRG1-associated congenital neutropenia. PMID: 24753205
    16. analysis demonstrates that CLCN7 and TCIRG1 mutations differentially affect bone matrix mineralization, and that there is a need to modify the current classification of osteopetrosis PMID: 24108692
    17. The function of vacuolar ATPase (V-ATPase) a subunit isoforms in invasiveness of MCF10a and MCF10CA1a human breast cancer cells. PMID: 24072707
    18. Our data highlights the importance of two large genomic deletions and mutations in the 5' UTR with respect to patient management and, more critically, to prenatal diagnosis PMID: 22231430
    19. The N termini of a-subunit isoforms are involved in signaling between vacuolar H+-ATPase (V-ATPase) and cytohesin-2 PMID: 23288846
    20. The novel mutation c.242delC of TCIRG1 in infantile malignant osteopetrosis PMID: 21042819
    21. V-ATPase localization and activity in kidney cells is regulated via direct PKA-dependent phosphorylation of the A subunit at Ser-175 PMID: 20525692
    22. That the CLCN7 mutations provoke a phenotype as severe as the one caused by TCIRG1 loss of function suggests the affected residues to be crucial for the function of the ClC-7 chloride channel or chloride/proton-exchanger PMID: 20424301
    23. localization to chromosome 11q12-13 in autosomal dominant osteopetrosis type I PMID: 12054167
    24. Sibling pair linkage and association studies between peak bone mineral density and the gene locus for the osteoclast-specific subunit (OC116) of the vacuolar proton pump on chromosome 11p12-13. PMID: 12161516
    25. Four novel single nucleotide mutations in the TCIRG1 gene encoding the 116-kDa osteoclast specific subunit of ATP6I affecting splice acceptor or donor sites result in aberrant transcription products. PMID: 12552563
    26. There is an association between a polymorphism affecting an API binding site in the promoter of the TCIRG1 gene and bone mass in Scottish women. PMID: 14523594
    27. 9 new TCIRG1 mutations were found in recessive osteopetrosis pts. 30% of the pts had c.1674-1G>A (aberrant splicing: r.1674_1884del) or c.2005C>T (protein variation: p.Arg669X). 40% were splicing regulatory sequence substitutions. PMID: 15300850
    28. we validated by RT-PCR six new alternative splice events in TCIRG1 in most of the 28 human tissues studied PMID: 15809087
    29. TIRC7 acts as an upstream regulatory molecule of cytotoxic T-lymphocyte antigen 4 (CTLA-4) expression. PMID: 17082597
    30. HLA-DR alpha 2 domain (sHLA-DRalpha2) induces negative signals by engaging TIRC7 on lymphocytes, inhibiting proliferation and inducing apoptosis in CD4+ and CD8+ T-cells via activation of the intrinsic pathway PMID: 18270567
    31. analysis of a novel Alu-Alu recombination-mediated genomic deletion in the TCIRG1 gene in five osteopetrotic patients PMID: 18715141
    32. Linkage disequilibrium (LD) mapping of the OPTB locus at the TCIRG1 region and found a unique splice site mutation c.807+5G>A in all Chuvashian OPTB patients studied. PMID: 19172990
    33. Mutations in TCIRG1, OSTM1, ClCN7, and TNFRSF11A genes were detected in nine, three, one, and one patientswith infantile malignant osteopetrosis, respectively. PMID: 19507210

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  • 相关疾病：
    Osteopetrosis, autosomal recessive 1 (OPTB1)
  • 亚细胞定位：
    Membrane; Multi-pass membrane protein.
  • 蛋白家族：
    V-ATPase 116 kDa subunit family
  • 组织特异性：
    Isoform long is highly expressed in osteoclastomas. Isoform short is highly expressed in thymus.
  • 数据库链接：

    HGNC: 11647

    OMIM: 259700

    KEGG: hsa:10312

    STRING: 9606.ENSP00000265686

    UniGene: Hs.495985