TCIRG1 Antibody, FITC conjugated

說明書

中文名稱：

TCIRG1兔多克隆抗體, FITC偶聯(lián)
貨號：

CSB-PA615690LC01HU
規(guī)格：

￥880
其他：

產(chǎn)品詳情
產(chǎn)品評價
相關(guān)產(chǎn)品
靶點詳情

產(chǎn)品詳情

產(chǎn)品名稱：

Rabbit anti-Homo sapiens (Human) TCIRG1 Polyclonal antibody
Uniprot No.：

Q13488
基因名：

TCIRG1
別名：

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
反應(yīng)種屬：

Human
免疫原：

Recombinant Human V-type proton ATPase 116 kDa subunit a isoform 3 protein (76-182AA)
免疫原種屬：

Homo sapiens (Human)
標(biāo)記方式：

FITC
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
產(chǎn)品提供形式：

Liquid
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(chǎn)品評價

靶點詳情

功能：

Part of the proton channel of V-ATPases. Seems to be directly involved in T-cell activation.
基因功能參考文獻(xiàn)：
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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相關(guān)疾病：

Osteopetrosis, autosomal recessive 1 (OPTB1)
亞細(xì)胞定位：

Membrane; Multi-pass membrane protein.
蛋白家族：

V-ATPase 116 kDa subunit family
組織特異性：

Isoform long is highly expressed in osteoclastomas. Isoform short is highly expressed in thymus.
數(shù)據(jù)庫鏈接：

HGNC: 11647

OMIM: 259700

KEGG: hsa:10312

STRING: 9606.ENSP00000265686

UniGene: Hs.495985

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