| 中文名稱 | 肌動蛋白α/α-SMA/α Actin抗體 |
| 別 名 | alpha sarcomeric Actin; alpha smooth muscle Actin; Actin alpha; ASMA; ASM-A; alpha-SMA; alpha SMA; AAT6; ACTA2; Actin alpha 2 smooth muscle aorta; Actin aortic smooth muscle; ACTSA; ACTVS; Alpha 2 actin; Alpha-actin 2; Cell growth inhibiting gene 46 protein; Growth inhibiting gene 46; ACTA_HUMAN; Actin alpha 2 smooth muscle aorta; Actin aortic smooth muscle; Actin, aortic smooth muscle; Alpha 2 actin; Alpha actin 2; Alpha cardiac actin; Alpha-actin 2; Alpha-actin-2; Cell growth inhibiting gene 46 protein; Cell growth-inhibiting gene 46 protein; Growth inhibiting gene 46; MYMY5 |
| 研究領域 | 腫瘤 細胞生物 免疫學 細胞骨架 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應 | Human, Mouse, Rat, Dog, HMt,Op (predicted: Chicken, Pig, Cow, Rabbit, Sheep, Fish, Guinea Pig, Hamster, Cat, ) |
| 產品應用 | WB=1:500-2000 ELISA=1:500-1000 Flow-Cyt=1μg /test not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 42kDa |
| 細胞定位 | 細胞漿 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Actin alpha:301-375/375 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
| PubMed | PubMed |
| 產品介紹 | The product encoded by this gene belongs to the actin family of proteins, which are highly conserved proteins that play a role in cell motility, structure and integrity. Alpha, beta and gamma actin isoforms have been identified, with alpha actins being a major constituent of the contractile apparatus, while beta and gamma actins are involved in the regulation of cell motility. This actin is an alpha actin that is found in skeletal muscle. Mutations in this gene cause nemaline myopathy type 3, congenital myopathy with excess of thin myofilaments, congenital myopathy with cores, and congenital myopathy with fiber-type disproportion, diseases that lead to muscle fiber defects. [provided by RefSeq, Jul 2008] Function: Actins are highly conserved proteins that are involved in various types of cell motility and are ubiquitously expressed in all eukaryotic cells. Subunit: Polymerization of globular actin (G-actin) leads to a structural filament (F-actin) in the form of a two-stranded helix. Each actin can bind to 4 others. Subcellular Location: Cytoplasm, cytoskeleton. Post-translational modifications: Oxidation of Met-46 by MICALs (MICAL1, MICAL2 or MICAL3) to form methionine sulfoxide promotes actin filament depolymerization. Methionine sulfoxide is produced stereospecifically, but it is not known whether the (S)-S-oxide or the (R)-S-oxide is produced (By similarity). DISEASE: Note=ACTA2 mutations predispose patients to a variety of diffuse and diverse vascular diseases, premature onset coronary artery disease (CAD), premature ischemic strokes and Moyamoya disease. Defects in ACTA2 are the cause of familial aortic aneurysm thoracic type 6 (AAT6) [MIM:611788]. AATs are characterized by permanent dilation of the thoracic aorta usually due to degenerative changes in the aortic wall. They are primarily associated with a characteristic histologic appearance known as 'medial necrosis' or 'Erdheim cystic medial necrosis' in which there is degeneration and fragmentation of elastic fibers, loss of smooth muscle cells, and an accumulation of basophilic ground substance. Defects in ACTA2 are the cause of Moyamoya disease type 5 (MYMY5) [MIM:614042]. Moyamoya disease is a progressive cerebral angiopathy characterized by bilateral intracranial carotid artery stenosis and telangiectatic vessels in the region of the basal ganglia. The abnormal vessels resemble a 'puff of smoke' (moyamoya) on cerebral angiogram. Affected individuals can develop transient ischemic attacks and/or cerebral infarction, and rupture of the collateral vessels can cause intracranial hemorrhage. Hemiplegia of sudden onset and epileptic seizures constitute the prevailing presentation in childhood, while subarachnoid bleeding occurs more frequently in adults. Defects in ACTA2 are the cause of multisystemic smooth muscle dysfunction syndrome (MSMDYS) [MIM:613834]. MSMDYS is a syndrome characterized by dysfunction of smooth muscle cells throughout the body, leading to aortic and cerebrovascular disease, fixed dilated pupils, hypotonic bladder, malrotation, and hypoperistalsis of the gut and pulmonary hypertension. Similarity: Belongs to the actin family. SWISS: P62736 Gene ID: 59 Database links: Entrez Gene: 101021287 Baboon Entrez Gene: 515610 Cow Entrez Gene: 59 Human Entrez Gene: 11475 Mouse Entrez Gene: 733615 Pig Entrez Gene: 100009271 Rabbit Entrez Gene: 81633 Rat Omim: 102620 Human SwissProt: P62739 Cow SwissProt: P62736 Human SwissProt: P62737 Mouse SwissProt: P62740 Rabbit SwissProt: P62738 Rat Unigene: 500483 Human Unigene: 213025 Mouse Unigene: 195319 Rat Unigene: 3114 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 結構蛋白（Structural Proteins） Actin α/α-Actin 是一種具有收縮能力的微絲蛋白，a-SMA廣泛分布于幾乎所有的肌型細胞中。Actin-α蛋白主要用于檢測骨骼肌、平滑肌、血管平滑肌、心肌和肌原性腫瘤 包括：平滑肌瘤、平滑肌肉瘤、橫紋肌肉瘤以及肌上細胞和肌上皮瘤。Actin（肌動蛋白）是在所有真核細胞中都表達的高度保守的蛋白質。它們沿微管組成了細胞骨架的主要成分。肌動蛋白至少表達為6種異構形式。它在心臟、骨骼橫紋肌組織和某些平滑肌組織中表達，調節其收縮功能。有報導說肌動蛋白在乳房瘤中是高度磷酸化的。肌動蛋白的功能失調也會導致某種類型的心臟病。平滑肌α肌動蛋白使人更感興趣，因為編碼它的基因是相對局限于在血管平滑肌細胞中表達的少數幾個基因之一。肌動蛋白是標記平滑肌和肌上皮細胞腫瘤的有效工具。 |
| 產品圖片 |  Sample:Kidney (Mouse) Lysate at 40 ugPrimary: Anti- alpha-SMA (bs-0189R) at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 42 kD Observed band size: 42 kD  Sample:Ovary (Mouse) Lysate at 40 ugPrimary: Anti-alpha-SMA (bs-0189R) at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 42 kD Observed band size: 42 kD  Sample: A549(Human) Lysate at 40 ugPrimary: Anti- alpha-SMA (bs-0189R) at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 42 kD Observed band size: 42 kD Sample: Brain (Rat) Lysate at 40 ug Kidney (Rat) Lysate at 40 ug Primary: Anti-alpha-SMA(bs-0189R) at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 42 kD Observed band size: 42 kD Sample: Brain (Rat) Lysate at 40 ug Kidney (Rat) Lysate at 40 ug Primary: Anti- alpha-SMA (bs-0189R) at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 42 kD Observed band size: 42 kD  Blank control (blue line): Hela (blue).Primary Antibody (green line): Rabbit Anti-alpha smooth muscle Actin antibody (bs-0189R) Dilution: 1μg /10^6 cells; Isotype Control Antibody (orange line): Rabbit IgG . Secondary Antibody (white blue line): Goat anti-rabbit IgG-FITC Dilution: 1μg /test. Protocol The cells were fixed with 80% methanol (5 min at -20℃) and then permeabilized with 0.1% PBS-Tween for 20 min at room temperature. Cells stained with Primary Antibody for 30 min at room temperature. The cells were then incubated in 1 X PBS/2%BSA/10% goat serum to block non-specific protein-protein interactions followed by the antibody for 15 min at room temperature. The secondary antibody used for 40 min at room temperature. Acquisition of 20,000 events was performed.  Blank control: NIH/3T3.Primary Antibody (green line): Rabbit Anti-alpha smooth muscle Actin antibody (bs-0189R) Dilution: 1μg /10^6 cells; Isotype Control Antibody (orange line): Rabbit IgG . Secondary Antibody : Goat anti-rabbit IgG-AF488 Dilution: 1μg /test. Protocol The cells were fixed with 4% PFA (10min at room temperature)and then permeabilized with 90% ice-cold methanol for 20 min at -20℃. The cells were then incubated in 5%BSA to block non-specific protein-protein interactions for 30 min at room temperature .Cells stained with Primary Antibody for 30 min at room temperature. The secondary antibody used for 40 min at room temperature. Acquisition of 20,000 events was performed. |
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