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Mouse Anti-alpha smooth muscle Actin/PE-Cy5 Conjugated antibody (bsm-33188M-PE-Cy5)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bsm-33188M-PE-Cy5
英文名稱 Mouse Anti-alpha smooth muscle Actin/PE-Cy5 Conjugated antibody
中文名稱 PE-Cy5標記的肌動蛋白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  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
產品類型 內參抗體 
研究領域 細胞生物  發育生物學  細胞骨架  
抗體來源 Mouse
克隆類型 Monoclonal
克 隆 號 8B2
交叉反應 Human, Mouse,  (predicted: Rat, )
產品應用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 42kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human alpha smooth muscle Actin
亞    型 IgG
純化方法 affinity purified by Protein G
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.
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 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.

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。
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