TCIRG1 Antibody

Code CSB-PA615690LA01HU
Size US$166
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  • 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).

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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) TCIRG1 Polyclonal antibody
Uniprot No.
Target Names
TCIRG1
Alternative Names
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
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human V-type proton ATPase 116 kDa subunit a isoform 3 protein (76-182AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated

The TCIRG1 Antibody (Product code: CSB-PA615690LA01HU) is Non-conjugated. For TCIRG1 Antibody with conjugates, please check the following table.

Available Conjugates
Conjugate Product Code Product Name Application
HRP CSB-PA615690LB01HU TCIRG1 Antibody, HRP conjugated ELISA
FITC CSB-PA615690LC01HU TCIRG1 Antibody, FITC conjugated
Biotin CSB-PA615690LD01HU TCIRG1 Antibody, Biotin conjugated ELISA
Clonality
Polyclonal
Isotype
IgG
Purification Method
>95%, Protein G purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form
Liquid
Tested Applications
ELISA, IHC, IF
Recommended Dilution
Application Recommended Dilution
IHC 1:200-1:500
IF 1:50-1:200
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
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.

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Target Background

Function
Part of the proton channel of V-ATPases. Seems to be directly involved in T-cell activation.
Gene References into Functions
  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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Involvement in disease
Osteopetrosis, autosomal recessive 1 (OPTB1)
Subcellular Location
Membrane; Multi-pass membrane protein.
Protein Families
V-ATPase 116 kDa subunit family
Tissue Specificity
Isoform long is highly expressed in osteoclastomas. Isoform short is highly expressed in thymus.
Database Links

HGNC: 11647

OMIM: 259700

KEGG: hsa:10312

STRING: 9606.ENSP00000265686

UniGene: Hs.495985

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