Human ADAMTS-like protein 4(ADAMTSL4) ELISA kit

中文名称：

人ADAMTS样蛋白4(ADAMTSL4)酶联免疫试剂盒
货号：

CSB-EL001320HU
规格：

96T/48T
价格：

￥3600/￥2500
其他：

CUSABIO Wins 2024 Bioz Rapid Star™ Award in the ELISA category

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人ADAMTS样蛋白4（ADAMTSL4）酶联免疫试剂盒（货号：CSB-EL001320HU）采用双抗体夹心法原理，可定量检测血清、血浆及组织匀浆样本中ADAMTSL4蛋白的表达水平。ADAMTSL4是ADAMTS蛋白家族成员之一，参与细胞外基质调控及蛋白质相互作用，其功能异常可能与眼部发育疾病及结缔组织病变相关。本试剂盒检测灵敏度高，线性范围为0.156 ng/mL至10 ng/mL，可精准检测低丰度样本，适用于基础科研中探索ADAMTSL4在组织发育、疾病机制或分子调控网络中的作用。实验操作采用预包被特异性抗体的96孔板，配套标准品与检测抗体，通过显色反应进行结果分析，支持手动或酶标仪自动化检测。该试剂盒兼容多种生物样本类型，为研究ADAMTSL4在体液中的表达动态或不同组织中的分布特征提供可靠工具，可应用于细胞实验、疾病模型研究或生物标志物筛选等非临床领域的科研需求。
别名：

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
缩写：

ADAMTSL4
Uniprot No.：

Q6UY14
种属：

Homo sapiens (Human)
样本类型：

serum, plasma, tissue homogenates
检测范围：

0.156 ng/mL-10 ng/mL
灵敏度：

0.039 ng/mL
反应时间：

1-5h
样本体积：

50-100ul
检测波长：

450 nm
研究领域：

Cell Biology
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 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

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
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

数据处理：

ELISA操作视频及标曲绘制软件下载
货期：

3-5 working days

引用文献

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.
基因功能参考文献：
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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相关疾病：

Ectopia lentis 2, isolated, autosomal recessive (ECTOL2); Ectopia lentis et pupillae (ECTOLP)
亚细胞定位：

Secreted, extracellular space, extracellular matrix. Note=Colocalizes with FMN1 microfibrils in the eye ECM.
组织特异性：

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
数据库链接：

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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