產品編號 yb-0189R
英文名稱 Anti-alpha smooth muscle Actin抗體
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
Anti-alpha smooth muscle Actin抗體
說 明 書 0.1ml  0.2ml  
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, Chicken, Dog, Pig, Cow, Rabbit, Sheep, Fish, Guinea Pig, Hamster, Cat, HMt,Op
產品應用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復） 
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 Lyophilized or Liquid
濃    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human Actin alpha C-terminus
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 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

PubMed PubMed
產品介紹 background:
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 angiectatic 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.

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。