Human ADAMTS-like protein 4(ADAMTSL4) ELISA kit

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中文名稱：

人ADAMTS樣蛋白4(ADAMTSL4)酶聯免疫試劑盒
貨號：

CSB-EL001320HU
規(guī)格：

96T/48T
價格：

￥3600/￥2500
其他：

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人ADAMTS樣蛋白4(ADAMTSL4)酶聯免疫試劑盒（CSB-EL001320HU）為雙抗夾心法ELISA試劑盒，定量檢測血清、血漿、組織勻漿樣本中的ADAMTSL4含量。ADAMTSL4是一種具有血小板反應蛋白基序的解聚素和金屬蛋白酶4，與系統(tǒng)性硬化癥（SSc）和肺動脈高壓（PAH）的預測和診斷相關。其研究機制涉及在SSc患者中檢測ADAMTSL4抗體水平，以預測PAH的發(fā)生可能性。試劑盒檢測范圍為0.156 ng/mL-10 ng/mL，適用于基礎科研中探索ADAMTSL4在組織發(fā)育、疾病機制或分子調控網絡中的作用；為研究ADAMTSL4在體液中的表達動態(tài)或不同組織中的分布特征提供可靠工具；可應用于細胞實驗、疾病模型研究或生物標志物篩選等非臨床領域的科研需求。本品僅用于科研，不用于臨床診斷，產品具體參數及操作步驟詳見產品說明書。
別名：

ADAMTSL4 ELISA kit; TSRC1 ELISA kit; PP1396 ELISA kit; UNQ2803/PRO34012ADAMTS-like protein 4 ELISA kit; ADAMTSL-4 ELISA kit; Thrombospondin repeat-containing protein 1 ELISA kit
縮寫：

ADAMTSL4
Uniprot No.：

Q6UY14
種屬：

Homo sapiens (Human)
樣本類型：

serum, plasma, tissue homogenates
檢測范圍：

0.156 ng/mL-10 ng/mL
靈敏度：

0.039 ng/mL
反應時間：

1-5h
樣本體積：

50-100ul
檢測波長：

450 nm
研究領域：

Cell Biology
測定原理：

quantitative
測定方法：

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.

線性度：

To assess the linearity of the assay, samples were spiked with high concentrations of human ADAMTSL4 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 %	92
	Range %	87-96
1:2	Average %	101
	Range %	98-104
1:4	Average %	85
	Range %	80-90
1:8	Average %	102
	Range %	98-106

回收率：

The recovery of human ADAMTSL4 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)	84	81-87
EDTA plasma (n=4)	96	93-99

標準曲線：

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
10	2.044	1.968	2.006	1.840
5	1.753	1.775	1.764	1.598
2.5	1.415	1.402	1.409	1.243
1.25	1.004	1.084	1.044	0.878
0.625	0.712	0.700	0.706	0.540
0.312	0.523	0.505	0.514	0.348
0.156	0.301	0.312	0.307	0.141
0	0.167	0.164	0.166

數據處理：

ELISA操作視頻及標曲繪制軟件下載
貨期：

3-5 working days

引用文獻

METHODS AND SYSTEM FOR DETERMINING THE DISEASE STATUS OF A SUBJECT Price, Andrew.et al,United States Application,2017

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功能：

Positive regulation of apoptosis. May facilitate FBN1 microfibril biogenesis.
基因功能參考文獻：
1. A recurrent pathogenic ADAMTSL4 variant is a major cause of early onset autosomal recessive ectopia lentis in a Cook Island Maori population and associated with a common haplotype, suggesting a founder effect. PMID: 28394649
2. study reports a novel founder mutation in ADAMTSL4 gene in children of Bukharian Jewish origin presenting with early-onset bilateral ectopia lentis PMID: 26653794
3. ADAMTSL4 mutations are the main cause for isolated ectopia lentis with an early onset of symptoms and possible severe ocular complications. PMID: 25975359
4. Compound heterozygous c.1783dupT and c. 2594G>A mutations of ADAMTSL4 gene were causative mutations for this family with isolated non-syndromic ectopia lentis PMID: 24802351
5. We have confirmed the gene and protein expression of ADAMTSL4 in human ocular tissue. The pattern of expression may suggest further functions of this gene beyond those suggested by its causative role in isolated ectopia lentis. PMID: 23846871
6. This is the first detailed report of a possible genetic determinant of craniosynostosis with ectopia lentis. PMID: 22871183
7. Patients from a family with ectopia lentis et pupillae (ELP) in four generations have autosomal recessive ELP caused by novel mutations in ADAMTSL4. PMID: 23426735
8. Mutations in ADAMTSL4 appear to cause earlier manifestation of ectopia lentis and are associated with increased axial length. PMID: 22736615
9. Enhanced fibrillin-1 deposition in the presence of ADAMTSL4 and colocalization of ADAMTSL4 with fibrillin-1 in the ECM of cultured fibroblasts suggest a potential role for ADAMTSL4 in the formation or maintenance of the zonule. PMID: 21989719
10. The results emphasize the association of ADAMTSL4 null mutations with isolated ectopia lentis and the presence of a founder mutation in the European population. PMID: 21051722
11. Ectopia lentis et pupillae is associated with a number of malformations primarily in the anterior segment of the eye. PMID: 20702823
12. This study confirms that homozygous mutations in ADAMTSL4 are associated with autosomal-recessive ectopia lentis in British families. PMID: 20564469
13. Herein we show a consanguineous family that carries a novel homozygous splice mutation IVS4-1G>A/IVS4-1G>A in ADAMTSL4 responsible for isolated autosomal recessive ectopia lentis. PMID: 20141359
14. Mutations in ADAMTSL4 are responsible for autosomal-recessive simple ectopia lentis and this proteins affectsthe development of the zonular fibers. PMID: 19200529
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相關疾?。?/div>
Ectopia lentis 2, isolated, autosomal recessive (ECTOL2); Ectopia lentis et pupillae (ECTOLP)
亞細胞定位：

Secreted, extracellular space, extracellular matrix. Note=Colocalizes with FMN1 microfibrils in the eye ECM.
組織特異性：

Expressed in colon, heart, leukocyte, liver, lung, skeletal muscle, spleen, testis and placenta. Weaker expression in bone marrow, brain tissue, kidney and pancreas. Expression studies in fetal tissues reveal strong expression in heart, kidney, liver, lun
數據庫鏈接：

HGNC: 19706

OMIM: 225100

KEGG: hsa:54507

STRING: 9606.ENSP00000271643

UniGene: Hs.516243

Human ADAMTS-like protein 4(ADAMTSL4) ELISA kit

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