ECM1 Recombinant Monoclonal Antibody

說明書

中文名稱：

ECM1 Recombinant Monoclonal Antibody
貨號：

CSB-RA215300A0HU
規格：

￥1320
圖片：
IHC image of CSB-RA215300A0HU diluted at 1:100 and staining in paraffin-embedded human breast cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB.

Immunofluorescence staining of Hela cell with CSB-RA215300A0HU at 1:50, 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).

Overlay Peak curve showing A375 cells stained with CSB-RA215300A0HU (red line) at 1:100. The cells were fixed in 4% formaldehyde and permeated by 0.2% TritonX-100 for?10min. Then 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (1ug/1*10⁶cells) for 45min at 4℃. The secondary antibody used was FITC-conjugated goat anti-rabbit IgG (H+L) at 1/200 dilution for 35min at 4℃.Control antibody (green line) was Rabbit IgG (1ug/1*10⁶cells) used under the same conditions. Acquisition of >10,000 events was performed.
其他：

產品詳情
產品評價
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靶點詳情

產品詳情

Uniprot No.：

Q16610
基因名：

ECM1
別名：

ECM 1 antibody; Ecm1 antibody; ECM1_HUMAN antibody; Extracellular matrix protein 1 antibody; Secretory component p85 antibody; URBWD antibody
反應種屬：

Human
免疫原：

A synthesized peptide derived from human ECM1
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
克隆類型：

Monoclonal
抗體亞型：

Rabbit IgG
純化方式：

Affinity-chromatography
克隆號：

5E12
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Rabbit IgG in 10mM phosphate buffered saline , pH 7.4, 150mM sodium chloride, 0.05% BSA, 0.02% sodium azide and 50% glycerol.
產品提供形式：

Liquid
應用范圍：

ELISA, IHC, IF, FC

推薦稀釋比：

Application	Recommended Dilution
IHC	1:50-1:200
IF	1:50-1:200
FC	1:50-1:200

Protocols：

ELISA Protocol
Immunohistochemistry (IHC) Protocol
Immunofluorescence (IF) Protocol
Flow cytometry (FC) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

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.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

功能：

Involved in endochondral bone formation as negative regulator of bone mineralization. Stimulates the proliferation of endothelial cells and promotes angiogenesis. Inhibits MMP9 proteolytic activity.
基因功能參考文獻：
1. the present study demonstrated the association between ECM1 gene mutation and patients with LP. Patients with LP exhibited one homozygous point mutation (C220G) as previously reported, one novel homozygous mutation (c.508insCTG) and two heterozygous mutations (C220G/P.R481X and c.507delT/c.l473delT). PMID: 29693130
2. The results showed a significant upregulation of ECM1 and ITGB3, and a significant downregulation for FBLN5 in pelvic organ prolapse patients. PMID: 29729708
3. Three patients with homozygous mutations in sixth and seventh exons of the ECM1 gene had a drug-resistant course at the end of long-term follow-up PMID: 28434238
4. This proteome analysis indicate that ECM1 is a potential novel plasma protein biomarker for the detection of primary ESCC and evaluation of neoplasms progression PMID: 28493612
5. The TT genotype of ECM1 gene rs3737240 SNP significantly increased susceptibility for Ulcerative Colitis and azathioprine use in Ulcerative Colitis patients in a Turkish population. PMID: 28699600
6. our work has identified a novel function of ECM1 in inhibiting Th17 cell differentiation in the experimental autoimmune encephalomyelitis model PMID: 27316685
7. Our results indicate that although mutation in ECM1gene is responsible for lipoid proteinosis, it is likely that this is not the only gene causing this disease and probably other genes may be involved in the pathogenesis of the LP disease. PMID: 27241643
8. ECM1, which displayed a high expression in hepatocellular carcinoma (HCC) specimens, was closely associated with clinicopathologic data and may promote migration and invasion of HCC cells by inducing epithelia-mesenchymal transition. PMID: 27460906
9. Cell invasion (matrigel) was reduced only in the Hs578T cells (p < 0.01). Silencing decreased the expression of the prometastatic molecules S100A4 and TGFbetaR2 in both cell lines and CD44 in Hs578T cells. We conclude that ECM1 is a key player in the metastatic process and regulates the actin cytoskeletal architecture of aggressive breast cancer cells at least in part via alterations in S100A4 and Rho A. PMID: 27770373
10. Overexpression of miR-486-3p inhibited cell growth and metastasis by targeting ECM1. PMID: 27133046
11. For 1q21 loci, we confirmed gene ECM1 as the most plausible gene from this region to be involved in pathogenesis of inflammatory bowel disease PMID: 26738999
12. In conclusion, the domain-specific anti-ECM1 MAbs produced in this study should provide a useful tool for investigating ECM1's biological functions, and cellular pathways in which it is involved. PMID: 26826312
13. Lipoid proteinosis is a rare autosomal recessive disorder caused by mutations in ECM1, encoding extracellular matrix protein 1, a glycoprotein expressed in many organs and which has important protein-protein interactions in tissue homeostasis PMID: 26564090
14. MMP-2 protein and ECM1 gene are useful preoperative markers for defining malignancy in suspicious thyroid nodules PMID: 25812648
15. Genetic testing of theECM1 gene showed a homozygous nonsense mutation c.1441C > T (p.Arg481X) in exon 10, confirming the diagnosis of lipoid proteinosis. PMID: 24079542
16. Lipoidproteinosis results from a large homozygous deletion of ECM1 gene in a Chinese family. PMID: 25518807
17. High extracellular matrix protein-1 expression is associated with the growth, metastasis and angiogenesis of laryngeal carcinoma PMID: 25824756
18. This large cohort revealed extensive phenotypic variability in individuals with the same mutation in ECM1. PMID: 25529926
19. Lipoid proteinosis (LP) is a rare autosomal recessive genodermatosis caused by mutations in extracellular matrix protein 1 (ECM1) that involves deposition of basement membrane-like material in the skin and other organs. PMID: 23534907
20. ECM1 induced the expression of genes that promote the Warburg effect, such as glucose transporter 1 (GLUT1), lactate dehydrogenase A (LDHA), and hypoxia-inducible factor 1 alpha (HIF-1alpha). PMID: 25446258
21. Report a global loss of 5hmC identified three new genes (ECM1, ATF5, and EOMES) with potential anti-cancer functions that may promote the understanding of the molecular mechanisms of hepatocellular carcinoma development and progression. PMID: 25517360
22. High extracellular matrix protein 1 expression is correlated to carcinogenesis and lymphatic metastasis of gastric cancer PMID: 24779890
23. The data supports the conclusion that the c.742G>T mutation nonsense mutation in ECM1 is the pathological cause of lipoid proteinosis. PMID: 24413997
24. homozygous missense mutation p.C220G of ECM1 was identified by Sanger sequencing, which is a major allele in Chinese patients with LP PMID: 24708644
25. splicing mutation in Chinese lipoid proteinosis family PMID: 23682690
26. Clinical assays for ECM1 and TEX101 have the potential to replace most of the diagnostic testicular biopsies and facilitate the prediction of outcome of sperm retrieval procedures, increasing the reliability and success of assisted reproduction techniques PMID: 24259048
27. role for TFAP2C in melanoma via its regulation of ECM1 PMID: 24023917
28. Suggest that ECM1 plays promotive roles in the occurrence, development and metastasis of laryngeal carcinoma. PMID: 23696932
29. The expression of ECM1 was found to be an independent factor for predicting overall and disease-free survival of hepatocellular carcinoma. PMID: 21128013
30. Overexpression of ECM1 contributes to migration and invasion in cholangiocarcinoma. PMID: 22489696
31. Case Report: a novel mutation in Pakistani family extends the body of evidence that supports the importance of ECM1 gene for the development of lipoid proteinosis. PMID: 21791056
32. neurologic and neuroradiologic characteristics and ECM1 gene mutations of seven individuals with lipoid proteinosis (LP) from three unrelated consanguineous families PMID: 21349189
33. ECM1 played an important role in the growth, metastasis and angiogenesis of laryngeal carcinoma. PMID: 16646403
34. PLSCR1 interacts with the tandem repeat region of ECM1a in the dermal epidermal junction zone of human skin. PMID: 20870722
35. The ECM/SULF1 and ECM/COLLAGEN metagenes showed inconsistent association with DMFS in the three prognostic data sets in both breast neoplasm subtypes, and the combined P values were not significant. PMID: 20805453
36. A novel homozygous 62-bp insertion in exon 8 of ECM1 in this Pakistani family is a rare mutation affecting both alleles and it may help in further understanding the multifunctional role of ECM1 PMID: 19519837
37. Extracellular matrix protein 1 gene (ECM1) mutations in lipoid proteinosis and genotype-phenotype correlation. Seven new homozygous nonsense or frameshift mutations. Exons 6 and 7 most common sites for ECM1 mutations. PMID: 12603844
38. These results indicate that ECM1 tends to be preferentially expressed by metastatic epithelial tumors. PMID: 14550953
39. Frther emphasizes the role of ECM-1 in lipoid proteinosis and highlights unresolved genotype-phenotype correlation in this disease. PMID: 16274456
40. it is reported here that ECM1 interacts with MMP9 and that such interactions diminish the proteolytic activity of MMP9 PMID: 16512877
41. We report here mutation analysis of the ECM1 gene in a Chinese family with lipoid proteinosis. PMID: 17721643
42. This article provides an update on the molecular pathology of lipoid proteinosis, including the addition of 15 new mutations in ECM1 to the mutation database [review] PMID: 17927570
43. ECM1 is a basement membrane protein of the skin PMID: 18200062
44. Single Nucleotide Polymorphism in ECM1 gene is associated with ulcerative colitis PMID: 18438406
45. A survey of ECM1 expression in different tumors indicated that ECM1, although not tumor specific, is significantly elevated in many malignant epithelial tumors that give rise to metastases, emphasizing its relevance in the cancer process. Review. PMID: 18443958
46. ECM1 variation was not associated with Crohn's disease. PMID: 19068216
47. ECM proteins such as EDBFN and collagen are upregulated in ERM and PDR, and are regulated by TGF-beta. PMID: 19219685
48. Functional and structural characterisation of human colostrum free secretory component. PMID: 19230975
49. ECM1 is a multifunctional binding core and/or a scaffolding protein interacting with a variety of extracellular and structural proteins, contributing to the maintenance of skin integrity and homeostasis. PMID: 19275936
50. Overexpression of ECM1 is associated with invasive breast carcinomas. PMID: 19521735
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Lipoid proteinosis (LiP)
亞細胞定位：

Secreted, extracellular space, extracellular matrix.
組織特異性：

Expressed in breast cancer tissues. Little or no expression observed in normal breast tissues. Expressed in skin; wide expression is observed throughout the dermis with minimal expression in the epidermis.
數據庫鏈接：

HGNC: 3153

OMIM: 247100

KEGG: hsa:1893

STRING: 9606.ENSP00000358045

UniGene: Hs.81071

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