ACTA1 Recombinant Monoclonal Antibody

說明書

中文名稱：

ACTA1重組抗體
貨號：

CSB-RA289956A0HU
規(guī)格：

￥1320
圖片：
IHC image of CSB-RA289956A0HU diluted at 1:50 and staining in paraffin-embedded human skeletal muscle tissue 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 0.72% DAB.

IHC image of CSB-RA289956A0HU diluted at 1:50 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 0.72% DAB.

Overlay Peak curve showing A549 cells stained with CSB-RA289956A0HU (red line) at 1:50. The cells were fixed in 4% formaldehyde and permeated by 0.2% TritonX-100. Then 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (1μg/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 (1μg/1*10⁶cells) used under the same conditions. Acquisition of >10,000 events was performed.
其他：

產(chǎn)品詳情
產(chǎn)品評價
相關(guān)產(chǎn)品
靶點詳情

產(chǎn)品詳情

產(chǎn)品描述：

CSB-RA289956A0HU重組單克隆抗體是針對ACTA1靶點研發(fā)的高特異性科研工具，適用于ELISA、免疫組化（IHC）和流式細胞術(shù)（FC）等多種實驗場景。ACTA1（肌動蛋白α1骨骼肌亞型）是細胞骨架的關(guān)鍵組成蛋白，廣泛參與肌肉收縮、細胞形態(tài)維持及遷移等生物學(xué)過程，其表達異常與多種肌肉相關(guān)疾病的發(fā)生發(fā)展密切相關(guān)。本抗體經(jīng)嚴格驗證，在IHC實驗中可清晰定位肌肉組織中的天然蛋白，推薦使用稀釋比例為1:50-1:200；在FC分析中能有效識別細胞表面或胞內(nèi)ACTA1抗原，建議采用相同稀釋范圍以確保檢測靈敏度。該產(chǎn)品適用于骨骼肌發(fā)育研究、肌源性腫瘤的分子標(biāo)記分析，以及肌纖維結(jié)構(gòu)功能異常等病理機制的體外實驗探索，為細胞骨架動態(tài)調(diào)控、肌肉退行性疾病模型構(gòu)建等基礎(chǔ)研究提供可靠的工具支持。
Uniprot No.：

P68133
基因名：

ACTA1
別名：

Actin, alpha skeletal muscle (Alpha-actin-1) [Cleaved into: Actin, alpha skeletal muscle, intermediate form], ACTA1, ACTA
反應(yīng)種屬：

Human
免疫原：

A synthesized peptide derived from Human ACTA1
免疫原種屬：

Homo sapiens (Human)
標(biāo)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：

4C1
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(chǎn)品提供形式：

Liquid
應(yīng)用范圍：

ELISA, IHC, FC

推薦稀釋比：

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

Protocols：

ELISA Protocol
Immunohistochemistry (IHC) 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(chǎn)品評價

靶點詳情

功能：

Actins are highly conserved proteins that are involved in various types of cell motility and are ubiquitously expressed in all eukaryotic cells.
基因功能參考文獻：
1. Our study demonstrates that high ActA level is an independent prognosis factor of survival in cancer patients. More than a basic marker of the severity of the neoplastic disease or of the inflammatory process, ActA seems to influence survival by contributing to the development of cachexia and loss of skeletal muscle mass. PMID: 28712119
2. The study confirmed ACTA1 mutations in four patients, including one with intranuclear rods, one with large intracytoplasmic aggregates, and two with nemaline intracytoplasmic rods. PMID: 28780987
3. Study shows that clinically severe ACTA1-related myopathy can present with muscle morphological findings suggestive of cytoplasmic body myopathy in the absence of definite nemaline rods. PMID: 28416349
4. Shorter than normal thin filament length contributes to the impaired force generation in patients with thin filament myopathy, but only in those who harbor specific mutations in NEB or ACTA1. PMID: 27074222
5. Over-expression of TNC, SMA, and vimentin were significantly correlated with the lower overall survival in prostate cancer patients. PMID: 28341124
6. ANA and ASMA evaluation in patients with liver transplantation and no history of autoimmune disease has no clinical relevance, since it varies in time and is not related to any risk factors or liver injury. Routine autoimmunity evaluation should be avoided. PMID: 28337446
7. Upon actin engagement, the N-terminal "strap" and helix 1 are displaced from the vinculin tail helical bundle to mediate actin bundling. PMID: 26493222
8. This study reported the new information on the frequency and phenotypes of congenital myopathy caused by ACTA1 mutations in subjects >/=5 years of age. PMID: 26172852
9. The authors propose that Lpd delivers Ena/VASP proteins to growing barbed ends and increases their actin polymerase activity by tethering them to actin filaments. PMID: 26295568
10. Mutations in ACTA1 can cause pathologic features consistent with myofibrillar myopathy. PMID: 25913210
11. TIMP-1 significantly increased levels of alpha-SMA. PMID: 25895516
12. novel homozygous recessive missense variant (c.460G>C, P.(Val154Leu)) was found in two brothers with infantile-onset congenital muscular dystrophy with rigid spine. PMID: 25182138
13. Myopathy associated with zebra bodies is part of the spectrum of myopathies associated with the ACTA1 gene. PMID: 25747004
14. New scapuloperoneal phenotype associated with an ACTA1 mutation. PMID: 25938801
15. Antisynthetase syndrome-associated myositis is characterized by distinctive myonuclear actin filament inclusions. PMID: 25746564
16. The predominant pathway mediated by Australian bat lyssavirus G envelope for internalization into HEK293T cells is clathrin-and actin-dependent also requiring Rab5. PMID: 24576301
17. Data show that the width and speed of the waves depend counter-intuitively on parameters, negative feedback, and the F-actin time scale. PMID: 23831272
18. Although most cases of severe infantile form of nemaline myopathy caused by ACTA1 mutations are sporadic and have no family history PMID: 24313005
19. These findings suggest that the direct contact of Dengue virus 2 E protein with 43 kDa actin protein may have a crucial function in Dengue virus 2 infection of ECV304 cells. PMID: 23376163
20. Myotonia in adult human skeletal actin transgenic mice may be explained on the basis of a mosaic expression of ClC-1 channels in different fibres and/or on alterations of other conductances. PMID: 23247112
21. Respiratory muscles from an animal model of nemaline myopathy with an ACTA1 mutation show distinct muscle fiber weakness compared to limb muscles. PMID: 23656990
22. The objective was to evaluate the presence and distribution of the lubricating and anti-adhesion glycoprotein lubricin and cells containing the contractile isoform smooth muscle alpha-actin (SMA) in pseudomembranes around loose hip prostheses. PMID: 23174700
23. analysis of the pattern of of evolutionarily conserved basic and acidic residues that constitutes the binding interface of actin-tropomyosin PMID: 23420843
24. Data show that GnRHR activation affected several cellular markers of locomotion, including actin organization and polymerization as well as active RhoA-GTP levels. PMID: 23176180
25. Data indicate that actin nucleation on Chlamydia inclusions exhibited moderate depolymerization dynamics. PMID: 23071671
26. The organization of actin and cytokeratin cytoskeleton and the expression of TCTP, p53,cyclin A, RhoA and actin in HIO180 non-transformed ovarian epithelial cells, and OVCAR3 and SKOV3 ovarian epithelial cancer cells, was studied. PMID: 23042265
27. Data show that the urinary messenger RNA (mRNA) levels of alpha-smooth muscle actin (alpha-SMA), fibronectin, and matrix metalloproteinase-9 (MMP-9) were significantly higher in the diabetic nephropathy (DN) group, and mRNA levels increased with DN progression. PMID: 21824169
28. High alpha-actin is associated with colorectal carcinogenesis. PMID: 21912905
29. Over-expression of Nkx2.5 and/or cardiac alpha-actin inhibit the contraction ability of adipose tissue-derived stromal cells-derived cardiomyocytes. PMID: 21691712
30. Platelets gal-1 forms an intracellular complex with monomeric actin. PMID: 22081313
31. Herpesviruses exploit actin and actin-associated myosin motors for viral entry and virion egress. [review] PMID: 21994736
32. CK2 regulates vaccinia virus dissemination and actin tail formation. PMID: 22209233
33. Actin playes differential roles in trafficking of Epstein-Barr virus in B cells and epithelial cells. PMID: 22031939
34. a link between extramuscular expression of alpha-skeletal muscle actin and clinical symptoms in non-skeletal muscle tissues of patients with ACTA1 mutations, and probably a functional role of alpha-skeletal muscle actin during fetal development PMID: 21514153
35. The filopodium: a stable structure with highly regulated repetitive cycles of elongation and persistence depending on the actin cross-linker fascin PMID: 21975552
36. Actin accumulates throughout the activated immunological synapse. PMID: 21931536
37. Actin-EGFP and ezrin-EGFP accumulated below pilus-coated beads when force was applied. PMID: 21340023
38. Data show that concurrent phosphorylation of cortactin by ERK1/2 and tyrosine kinases enables cells with the ability to regulate actin dynamics. PMID: 21079800
39. BCL2 interaction with actin in vitro may inhibit cell motility by enhancing actin polymerization PMID: 20716950
40. Results suggest that actin polymerization and bundling by VASP are critical for spine formation, expansion, and modulating synaptic strength. PMID: 20826790
41. Centrosomal actin was detected with the anti-actin antibody 1C7 that recognizes antiparallel ("lower dimer") actin dimers. PMID: 21108927
42. These findings demonstrated that PI3K-mediated actin rearrangements are required for Cronobacter sakazakii invasion of human brain microvascular endothelial cells. PMID: 20809254
43. Results confirm that DNaseI-binding loop (D-loop) is involved in stabilization of skeletal muscle actin structure, both as monomers and filaments; actin is stabilized by ligands (by phalloidin, aluminum fluoride, & ATP [and to lesser extent by ADP]). PMID: 20718862
44. Simultaneous tracking of both TCR clusters and GFP-actin speckles reveals their dynamic association and individual flow patterns. Actin retrograde flow directs the inward transport of TCR clusters PMID: 20686692
45. We conclude that the Drosophila indirect flight muscles provide a good model system for studying ACTA1 mutations PMID: 20452215
46. Data demonstrate for the first time that L-plastin contributes to the fine-tuning of actin turn-over, an activity which is regulated by Ser5 phosphorylation promoting its high affinity binding to the cytoskeleton. PMID: 20169155
47. Podocyte BK(Ca) channels are regulated by synaptopodin, Rho, and actin microfilaments. PMID: 20630939
48. these data identify NHS as a new regulator of actin remodelling. PMID: 20332100
49. ACTA1 mutations are involved in fiber size disproportion in congenital myotonic dystrophy PMID: 20179953
50. First insights of S100A10 function as a regulator of the filamentous actin network. PMID: 20100475
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相關(guān)疾病：

Nemaline myopathy 3 (NEM3); Myopathy, actin, congenital, with excess of thin myofilaments (MPCETM); Myopathy, congenital, with fiber-type disproportion (CFTD); Myopathy, scapulohumeroperoneal (SHPM)
亞細胞定位：

Cytoplasm, cytoskeleton.
蛋白家族：

Actin family
數(shù)據(jù)庫鏈接：

HGNC: 129

OMIM: 102610

KEGG: hsa:58

STRING: 9606.ENSP00000355645

UniGene: Hs.1288

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