肌動(dòng)蛋白α/α-SMA/α Actin抗體

規(guī)格：1mg/1ml

英文名： alpha smooth muscle Actin

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 act

分子量： 42kDa

儲(chǔ)存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce

克隆類型：Polyclonal

亞型：IgG

純化方法：affinity purified by Protein A

**原：KLH conjugated synthetic peptide derived from human Actin al

交叉反應(yīng)：Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Rabbit, Sheep, Fish, Guinea Pig, Hamster, Cat, HMt,Op

肌動(dòng)蛋白α/α-SMA/α Actin抗體細(xì)胞定位：細(xì)胞漿

產(chǎn)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 肌動(dòng)蛋白α/α-SMA/α Actin抗體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 *****s. 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. Gene ID: 59 Database links: Entrez Gene: 101021287 Baboon Entrez 肌動(dòng)蛋白α/α-SMA/α Actin抗體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(jié)構(gòu)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(xì)胞中。Actin-α蛋白主要用于檢測(cè)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(xì)胞和肌上皮瘤。Actin（肌動(dòng)蛋白）是在所有真核細(xì)胞中都表達(dá)的高度保守的蛋白質(zhì)。它們沿微管組成了細(xì)胞骨架的主要成分。肌動(dòng)蛋白至少表達(dá)為6種異構(gòu)形式。它在心臟、骨骼橫紋肌組織和某些平滑肌組織中表達(dá)，調(diào)節(jié)其收縮功能。有報(bào)導(dǎo)說(shuō)肌動(dòng)蛋白在**瘤中是高度磷酸化的。肌動(dòng)蛋白的功能失調(diào)也會(huì)導(dǎo)致某種類型的心臟病。平滑肌α肌動(dòng)蛋白使人更感興趣，因?yàn)榫幋a它的基因是相對(duì)局限于在血管平滑肌細(xì)胞中表達(dá)的少數(shù)幾個(gè)基因之一。肌動(dòng)蛋白是標(biāo)記平滑肌和肌上皮細(xì)胞腫瘤的有效工具。

肌動(dòng)蛋白α/α-SMA/α Actin抗體產(chǎn)品應(yīng)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(fù)） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.

研究領(lǐng)域：腫瘤  細(xì)胞生物  **學(xué)  細(xì)胞骨架  

儲(chǔ)存條件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

來(lái)源： Rabbit

外觀： Lyophilized or Liquid

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