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  4. Human Adenosine deaminase CECR1(CECR1) ELISA kit

Human Adenosine deaminase CECR1(CECR1) ELISA kit

  • 中文名称：
    人腺苷脱氨酶CECR1(CECR1)酶联免疫试剂盒
  • 货号：
    CSB-EL005187HU
  • 规格：
    96T/48T
  • 价格：
    ￥3600/￥2500
  • 其他：

产品详情

  • 产品描述：

    This Human CECR1 ELISA Kit was designed for the quantitative measurement of Human CECR1 protein in serum, plasma, tissue homogenates. It is a Sandwich ELISA kit, its detection range is 0.312 ng/mL-20 ng/mL and the sensitivity is 0.078 ng/mL.

  • 别名：
    ADA2 ELISA kit; ADGF ELISA kit; CECR1 ELISA kit; IDGFLAdenosine deaminase 2 ELISA kit; EC 3.5.4.4 ELISA kit; Cat eye syndrome critical region protein 1 ELISA kit
  • 缩写：
    CECR1
  • Uniprot No.：
  • 种属：
    Homo sapiens (Human)
  • 样本类型：
    serum, plasma, tissue homogenates
  • 检测范围：
    0.312 ng/mL-20 ng/mL
  • 灵敏度：
    0.078 ng/mL
  • 反应时间：
    1-5h
  • 样本体积：
    50-100ul
  • 检测波长：
    450 nm
  • 研究领域：
    Epigenetics and Nuclear Signaling
  • 测定原理：
    quantitative
  • 测定方法：
    Sandwich
  • 精密度：
    Intra-assay Precision (Precision within an assay): CV%<8%
    Three samples of known concentration were tested twenty times on one plate to assess.
    Inter-assay Precision (Precision between assays): CV%<10%
    Three samples of known concentration were tested in twenty assays to assess.
  • 线性度：
    To assess the linearity of the assay, samples were spiked with high concentrations of human CECR1 in various matrices and diluted with the Sample Diluent to produce samples with values within the dynamic range of the assay.
    　 Sample Serum(n=4)
    1:1 Average % 105
    Range % 101-109
    1:2 Average % 92
    Range % 88-96
    1:4 Average % 89
    Range % 84-93
    1:8 Average % 96
    Range % 91-100
  • 回收率：
    The recovery of human CECR1 spiked to levels throughout the range of the assay in various matrices was evaluated. Samples were diluted prior to assay as directed in the Sample Preparation section.
    Sample Type Average % Recovery Range
    Serum (n=5) 87 82-91
    EDTA plasma (n=4) 93 89-97
  • 标准曲线：
    These standard curves are provided for demonstration only. A standard curve should be generated for each set of samples assayed.
    ng/ml OD1 OD2 Average Corrected
    20 2.463 2.518 2.491 2.307
    10 1.954 1.894 1.924 1.740
    5 1.115 1.160 1.138 0.954
    2.5 0.716 0.679 0.698 0.514
    1.25 0.401 0.427 0.414 0.230
    0.625 0.279 0.291 0.285 0.101
    0.312 0.227 0.235 0.231 0.047
    0 0.182 0.186 0.184 　
  • 数据处理：
  • 货期：
    3-5 working days

产品评价

靶点详情

  • 功能：
    Adenosine deaminase that may contribute to the degradation of extracellular adenosine, a signaling molecule that controls a variety of cellular responses. Requires elevated adenosine levels for optimal enzyme activity. Binds to cell surfaces via proteoglycans and may play a role in the regulation of cell proliferation and differentiation, independently of its enzyme activity.
  • 基因功能参考文献：
    1. Adenosine deaminase type 2 deficiency with a homozygous premature stop codon masquerading as GATA2 deficiency: successful haploidentical sibling hematopoietic stem cell transplantation. PMID: 27130863
    2. Deficiency of ADA2 causes IgA-IgG antibody deficiencies in a family. PMID: 26922074
    3. High CECR1 activity is associated with brain tumor. PMID: 28453746
    4. Data show that 2 patients required a second transplant for engraftment failure were discovered to carry the deleterious ADA2 adenosine deaminase (CECR1) mutations and be ADA2 deficient. PMID: 28974505
    5. CECR1 function in (M2-like) macrophages mediates cross talk between macrophages and pericytes in GBM via paracrine PDGFB-PDGFRbeta signaling, promoting pericyte recruitment and migration, and tumor angiogenesis. PMID: 28534507
    6. We discuss three newly described monogenic autoinflammatory diseases [deficiency of adenosine deaminase 2 (DADA2), a subtype of macrophage activation syndrome (MAS), and stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI)], discuss the possibilities of somatic mosaicism and digenic inheritance, and give an update on new concepts in pathways involved in familial Mediterranean fever PMID: 27362340
    7. Findings indicate that ADA2 deficiency presents not only with vasculopathy but also with an immunodeficiency of the B cell compartment. Therefore, patients with antibody deficiency should be screened for ADA2 deficiency. PMID: 28493328
    8. The clinical manifestations of adenosine deaminase 2 deficiency ranged in severity from limited cutaneous involvement to severe multisystemic vasculitis; one-third of our cases (5 of 15) were currently asymptomatic, and required close monitoring. We recommend CECR1 screening for unaffected siblings of index cases, cases of familial vasculitis, and cases of PAN that is resistant to standard treatment PMID: 27059682
    9. Deficiency of Adenosine Deaminase 2 (DADA2) is a new autoinflammatory disease secondary to autosomal recessive mutations of CECR1 (Cat Eye Syndrome Chromosome Region 1) gene, mapped to chromosome 22q11.1. PMID: 27609179
    10. IL-17 receptor A and adenosine deaminase 2 deficiency due to deletion mutations was found in siblings with chronic mucocutaneous candidiasis and chronic systemic inflammation. PMID: 26607704
    11. This case series revealed large phenotypic variability in patients with ADA2 deficiency though they were homozygous for the same R169Q mutation in CECR1. PMID: 26867732
    12. Ectopic expression of miR-146b-3p suppressed ADA2 expression, activity, and TNF-alpha release in the AGA-treated human macrophages PMID: 25815338
    13. In patients with unexplained young-onset lacunar stroke accompanied by systemic inflammation and a positive family history suspicious of recessively inherited disease, ADA2 deficiency needs to be considered in the differential diagnosis. PMID: 25888558
    14. ADA2 (CECR1) may act as a regulator of neutrophil activation, and that a reduction of ADA2 activity results in significant endothelial damage via a neutrophil-driven process. PMID: 25278816
    15. We report on a 5-year-old girl with a severe vasculopathy who carried two novel mutations in CECR1. PMID: 24737293
    16. Loss-of-function mutations in CECR1 were associated with a spectrum of vascular and inflammatory phenotypes, ranging from early-onset recurrent stroke to systemic vasculopathy or vasculitis. PMID: 24552284
    17. Recessive loss-of-function mutations of ADA2, a growth factor that is the major extracellular adenosine deaminase, can cause polyarteritis nodosa vasculopathy with highly varied clinical expression. PMID: 24552285
    18. Increased ADA2 expression and activity are identified in human and porcine retinas with diabetes. PMID: 23685153
    19. Plasma AD2 may have a role in determining tumour response to treatment. PMID: 22558798
    20. The crystal structures of ADA2 and ADA2 bound to a transition state analogue presented here reveal the structural basis of the catalytic/signaling activity of adenosine deaminase growth factor/ADA2 proteins. PMID: 20147294
    21. findings show ADA2 is encoded by the CECR1 gene & belongs to novel family of ADA-related growth factors;ADA2 may be active in sites of inflammation during hypoxia & in areas of tumour growth where adenosine is elevated & extracellular pH is acidic PMID: 15926889

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  • 相关疾病：
    Polyarteritis nodosa (PAN); Sneddon syndrome (SNDDS)
  • 亚细胞定位：
    Secreted.
  • 蛋白家族：
    Metallo-dependent hydrolases superfamily, Adenosine and AMP deaminases family, ADGF subfamily
  • 组织特异性：
    Detected in blood plasma (at protein level). Widely expressed, with most abundant expression in human adult heart, lung, lymphoblasts, and placenta as well as fetal lung, liver, and kidney. In embryo, expressed in the outflow tract and atrium of the devel
  • 数据库链接：

    HGNC: 1839

    OMIM: 182410

    KEGG: hsa:51816

    STRING: 9606.ENSP00000262607

    UniGene: Hs.170310