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GPR98 Antibody

  • 货号：
    CSB-PA23089A0Rb
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Immunofluorescence staining of Hela cells with CSB-PA23089A0Rb at 1:166, 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) GPR98 Polyclonal antibody
  • Uniprot No.：
    Q8WXG9
  • 基因名：
    GPR98
  • 别名：
    DKFZp761P0710 antibody; FEB 4 antibody; FEB4 antibody; G protein coupled receptor 98 antibody; G-protein coupled receptor 98 antibody; GPR 98 antibody; GPR98 antibody; GPR98_HUMAN antibody; KIAA0686 antibody; MASS 1 antibody; Monogenic audiogenic seizure susceptibility 1 homolog antibody; Monogenic audiogenic seizure susceptibility protein 1 homolog antibody; USH 2B antibody; USH 2C antibody; USH2B antibody; USH2C antibody; Usher syndrome 2C antibody; Usher syndrome type 2C protein antibody; Usher syndrome type-2C protein antibody; Very large G protein coupled receptor antibody; Very large G protein coupled receptor 1 antibody; Very large G-protein coupled receptor 1 antibody; VLGR 1 antibody; VLGR 1b antibody; VLGR1 antibody; VLGR1b antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human G-protein coupled receptor 98 protein (6155-6306AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，GPR98 Antibody (CSB-PA23089A0Rb)，的标记方式是Non-conjugated。对于GPR98 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-PA23089B0Rb GPR98 Antibody, HRP conjugated ELISA
    FITC CSB-PA23089C0Rb GPR98 Antibody, FITC conjugated
    Biotin CSB-PA23089D0Rb GPR98 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, IF
  • 推荐稀释比：
    Application Recommended Dilution
    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：
    G-protein coupled receptor which has an essential role in the development of hearing and vision. Couples to G-alpha(i)-proteins, GNAI1/2/3, G-alpha(q)-proteins, GNAQ, as well as G-alpha(s)-proteins, GNAS, inhibiting adenylate cyclase (AC) activity and cAMP production. Required for the hair bundle ankle formation, which connects growing stereocilia in developing cochlear hair cells of the inner ear. In response to extracellular calcium, activates kinases PKA and PKC to regulate myelination by inhibiting the ubiquitination of MAG, thus enhancing the stability of this protein in myelin-forming cells of the auditory pathway. In retina photoreceptors, the USH2 complex is required for the maintenance of periciliary membrane complex that seems to play a role in regulating intracellular protein transport. Involved in the regulation of bone metabolism.; Cleaved ADGRV1 beta-subunit couples with G-alpha(i)-proteins, GNAI1/2/3, and constitutively inhibits adenylate cyclase (AC) activity with a stronger effect than full ADGRV1.
  • 基因功能参考文献：
    1. Data suggest that the ADGRV1 variation contributes to epilepsy with myoclonic seizures, although the inheritance pattern may be complex in many cases. In patients with 5q14.3 deletion and epilepsy, ADGRV1 haploinsufficiency likely contributes to seizure development. PMID: 29266188
    2. we found new causative mutations in heterozygous compound state, one missense and one nonsense mutation in the GPR98 gene in three deaf sibs. The first mutation located in exon7, corresponds to c.1054C > A, which causes a proline to threonine change at position 352 of the protein (p.Pro352Thr). The second mutation located within exon77 is c.16544delT that leads to a stop codon at position 5515of the protein (p.Leu5515*). PMID: 28951997
    3. 7 patients clinically classified as having USH2, genetic tests confirmed the USH2 diagnosis in 5 cases. Of these, 4 patients showed mutations in the USH2A gene and 1 patient in the ADGRV1 gene. The mutation of the ADGRV1/GPR98 gene has an extremely rare incidence and is associated with a diagnosis of USH type 2C. PMID: 28653555
    4. We identified two novel truncation mutations in GPR98 causing Usher syndrome. PMID: 26432996
    5. our results suggest that low expression of VLGR1 is a significant risk factor of epileptic seizures in patients with low-grade glioma PMID: 25511798
    6. Our findings also expand the spectrum of GPR98 mutations in USH and demonstrate that the long isoform of GPR98 might carry even more mutations of the GPR98 gene. PMID: 25572244
    7. Diagnosis of Usher Syndrome 2 caused by GPR98 mutations in advance of visual defects in the cohort of nonsyndromic HL patients highlights importane of genetic testing in the diagnosis. PMID: 25743181
    8. identified an independent Galphai signaling pathway of the VLGR1 beta-subunit and its regulatory mechanisms that may have a role in the development of Usher syndrome PMID: 24962568
    9. In Spain, USH2A and GPR98 are responsible for 95.8% and 5.2% of Usher syndrome 2 mutated cases, respectively. DFNB31 plays a minor role in the Spanish population. There was a group of patients in whom no mutation was found. PMID: 23441107
    10. genetic association studies in postmenopausal Japanese women: Study found association between an SNP in GPR98 (rs10514346) and bone mineral density in this population; data suggest that Gpr98 signaling pathway regulates bone metabolism. PMID: 22419726
    11. Mutation found in USH2A, GPR98, or DFNB31 account for the vast majority of USH2 patients and their analysis provide a robust pathway for routine molecular diagnosis. PMID: 22147658
    12. A nonsense mutation (S2652X) causing a deletion of the C-terminal 126 amino acid residues was identified in one family with febrile and afebrile seizures. PMID: 12402266
    13. USH2C and USH2A manifest photoreceptor disease with rod- and cone-mediated visual losses and thinning of the outer nuclear layer. PMID: 15671307
    14. GPR98 genes and the phenotypic heterogeneity and particularly the severe ocular affection first observed in one Usher syndrome patient. PMID: 18854872
    15. study describes for the first time two male patients with Usher syndrome type 2 with novel GPR98 mutations PMID: 19357117
    16. has seven copies of the EPTP repeat, a unifying protein sequence motif of a heterogenous group of proteins linked to epileptic diseases. The EPTP repeat probably forms a beta-propeller structure. PMID: 12217514

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  • 相关疾病：
    Usher syndrome 2C (USH2C); Febrile seizures, familial, 4 (FEB4)
  • 亚细胞定位：
    Cell membrane; Multi-pass membrane protein. Cell projection, stereocilium membrane. Photoreceptor inner segment.
  • 蛋白家族：
    G-protein coupled receptor 2 family, LN-TM7 subfamily
  • 组织特异性：
    Expressed at low levels in adult tissues.
  • 数据库链接：

    HGNC: 17416

    OMIM: 602851

    KEGG: hsa:84059

    STRING: 9606.ENSP00000384582

    UniGene: Hs.591777