Human Biotinidase(BTD) ELISA kit
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中文名稱:人生物素酰胺酶(BTD)酶聯(lián)免疫試劑盒
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貨號:CSB-EL002854HU
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規(guī)格:96T/48T
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價格:¥3600/¥2500
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其他:
產(chǎn)品詳情
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產(chǎn)品描述:人生物素酰胺酶(BTD)酶聯(lián)免疫試劑盒(CSB-EL002854HU)為競爭法ELISA試劑盒,定量檢測血清、血漿、組織勻漿樣本中的BTD含量。BTD是一個重要靶點。它在細(xì)胞的生理過程中發(fā)揮著一定作用。當(dāng)前研究主要聚焦其在相關(guān)信號通路中的作用機制,探尋與疾病發(fā)生發(fā)展的關(guān)聯(lián),有望為開發(fā)針對BTD的靶向藥物、攻克相關(guān)疾病提供理論依據(jù)。試劑盒檢測范圍為0.41 ng/ml- 300 ng/ml,靈敏度為0.41 ng/ml。研究者可通過定量分析不同樣本中BTD的表達水平,應(yīng)用于生物素代謝機制研究、遺傳性代謝疾病模型構(gòu)建、營養(yǎng)干預(yù)效果評估等基礎(chǔ)研究場景,為探索BTD在細(xì)胞代謝調(diào)控中的作用提供可靠工具本品僅用于科研,不用于臨床診斷,產(chǎn)品具體參數(shù)及操作步驟詳見產(chǎn)品說明書。
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別名:Biotinase ELISA Kit; Biotinidase ELISA Kit; Btd ELISA Kit; BTD_HUMAN ELISA Kit; EC 3.5.1.12 ELISA Kit
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縮寫:BTD
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Uniprot No.:
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種屬:Homo sapiens (Human)
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樣本類型:serum, plasma, tissue homogenates
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檢測范圍:0.41 ng/ml - 300 ng/ml
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靈敏度:0.41 ng/ml
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反應(yīng)時間:1-5h
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樣本體積:50-100ul
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檢測波長:450 nm
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研究領(lǐng)域:Metabolism
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測定原理:quantitative
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測定方法:Competitive
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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.
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線性度:
To assess the linearity of the assay, samples were spiked with high concentrations of human BTD 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 %
99
Range %
92-106
1:2
Average %
97
Range %
91-105
1:4
Average %
96
Range %
89-103
1:8
Average %
101
Range %
94-108
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回收率:
The recovery of human BTD 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)
92
86-98
EDTA plasma (n=4)
101
94-108
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標(biāo)準(zhǔn)曲線:
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
300
2.746
2.634
2.690
2.529
100
2.362
2.267
2.314
2.154
33.33
1.652
1.628
1.640
1.480
11.11
0.933
0.929
0.931
0.770
3.7
0.663
0.641
0.652
0.491
1.23
0.438
0.453
0.445
0.285
0.41
0.259
0.254
0.256
0.096
0
0.156
0.165
0.161
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數(shù)據(jù)處理:
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貨期:3-5 working days
相關(guān)產(chǎn)品
靶點詳情
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功能:Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation.
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基因功能參考文獻:
- BTD mutation is associated with biotinidase deficiency. PMID: 29995633
- Biotinidase deficiency is reviewed. PMID: 26577040
- Four rare missense variants were identified (ACTBL2 rs73757391 (5q11.2), BTD rs200337373 (3p25.1), KRT13 rs150321809 (17q21.2) and MC2R rs104894658 (18p11.21)), but only MC2R rs104894668 had a large effect size (OR = 9.66). PMID: 27378695
- The history and genetic basis of biotinidase deficiency has been presented. (Review) PMID: 26456103
- 48 novel alterations in the biotinidase gene have been identified; correlating the individual's serum enzymatic activity with genotype, were able to determine the effect of the novel alteration on enzyme activity and, thereby, determine its likelihood of being pathogenic in 44 of these individuals PMID: 26810761
- The common biotinidase gene mutations (p.R157H, p.D444H, c.98-104del7ins3, p.T532M) cumulatively accounted for 72.3% of all the mutant alleles in the Turkish population. PMID: 25754625
- Summary of the demographic features of patients identified as biotinidase deficient from August of 2012 through August of 2013 and mutation analysis results for 20 cases in the southeast region of Turkey. PMID: 25423671
- Three novel pathogenic variants in BTD gene were identified in a cohort of Brazilian patients with biotinidase deficiency and control suggesting an allelic heteregeneity of the condition. PMID: 25174816
- Report incidence of profound biotinase deficiency in Swedish newborns and adoptive immigrant children. PMID: 20224900
- High frequencies of biotinidase mutations may explain the high incidence of biotinidase deficiency in Hungary. PMID: 20549359
- Mutation analysis revealed three novel mutations, c.del631C and c.1557T>G within exon 4 and c.324-325insTA in exon 3 in Biotinidase deficiency patients and families. PMID: 23481307
- Four Somali patients have the P497S mutation, with one of the four being homozygous for the mutation PMID: 19757147
- Six different mutations in the biotinidase gene are identified in biotinidase in four Chinese patients; determination of biotinidase activities are performed for selective screening of biotinidase deficiency PMID: 19728141
- loss of overall biotinidase expression is a novel marker for thyroid cancer aggressiveness. PMID: 22911723
- Plasma BTD activity increases in hepatic glycogen storage disease patients. PMID: 20532819
- 140 known mutations in the biotinidase gene (BTD) that cause biotinidase deficiency, are reported. PMID: 20556795
- Mutations in biotinidase is associated with biotinidase deficiency. PMID: 20539236
- 12 patients with multiple carboxylase deficiency, six mutations were found in the BT gene and 4 in the HLCS gene, including 5 novel mutations. PMID: 19806568
- review of mutations causing biotinidase deficiency PMID: 11668630
- report of 17 novel mutations that cause profound biotinidase deficiency. Six of the mutations are due to deletions, whereas the remaining 11 mutations are missense mutations located throughout the gene PMID: 12359137
- analysis of mutations in biotinidase deficiency PMID: 15776412
- 21 different mutations were identified in 49 patients, including four novel mutations. Ten mutations proved to be unique to the Hungarian population. PMID: 17185019
- Posttranslational modification of histones by biotinylation can be catalyzed by biotinidase; role of this function is ambiguous. PMID: 18479898
- This case indicates that biotinidase deficiency should be included in the differential diagnosis of subacute myelopathy and emphasizes the importance of a prompt diagnosis to prevent irreversible neurological damage. PMID: 18645204
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相關(guān)疾?。?/div>Biotinidase deficiency (BTD deficiency)亞細(xì)胞定位:Secreted, extracellular space.蛋白家族:Carbon-nitrogen hydrolase superfamily, BTD/VNN family數(shù)據(jù)庫鏈接:
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