中/英文名称：

肌动蛋白α/α-SMA/α Actin抗体/ Anti- alpha smooth muscle Actin

别名：

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 cardiac 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

产品编号：

zy10018

规格：

0.1ml    0.2ml

抗体来源：

Rabbit

浓    度：

1mg/ml

缓冲液：

0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1%   Sodium azide；Reconstitute with 0.1ml sterile distilled water.

克隆类型：

Polyclonal

亚 型：

IgG

免 疫 原：

KLH conjugated synthetic peptide derived from   human Actin alpha C-terminus

交叉反应：

Human, Mouse, Rat, Chicken, Dog, Pig, Cow,   Rabbit, Sheep, Fish, Guinea Pig, Hamster, Cat, HMt,Op

状态：

Lyophilized or Liquid

分 子 量：

Predicted molecular weight 42kDa

纯化方法：

affinity purified by Protein A

运输与储存

运输:

The product is shipped at ambient temperature.   Upon receipt, store it immediately at the temperature recommended below.

储存:

Use a manual defrost freezer and avoid repeated   freezethaw cycles.

1 month from date of receipt, 2 to 8℃, reconstituted.

6 months from date of receipt, -20 to -70℃, reconstituted.

12 months from date of receipt, -20 to -70℃as supplied.

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.

产品应用

应用:

WB=1:100-500    ELISA=1:500-1000    IP=1:20-100    IHC-P=1:100-500  IHC-F=1:100-500  IF=1:100-500
  Not yet tested in other applications.
  Optimal working dilutions must be determined by the end user.

背景:

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]

其他:

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.

Gene   ID:

59

结构蛋白（Structural Proteins）

Actin α/α-Actin 是一种具有收缩能力的微丝蛋白，a-SMA广泛分布于几乎所有的肌型细胞中。Actin-α蛋白主要用于检测骨骼肌、平滑肌、血管平滑肌、心肌和肌原性肿瘤 包括：平滑肌瘤、平滑肌肉瘤、横纹肌肉瘤以及肌上细胞和肌上皮瘤。Actin（肌动蛋白）是在所有真核细胞中都表达的高度保守的蛋白质。它们沿微管组成了细胞骨架的主要成分。肌动蛋白至少表达为6种异构形式。它在心脏、骨骼横纹肌组织和某些平滑肌组织中表达，调节其收缩功能。有报导说肌动蛋白在乳房瘤中是高度磷酸化的。肌动蛋白的功能失调也会导致某种类型的心脏病。平滑肌α肌动蛋白使人更感兴趣，因为编码它的基因是相对局限于在血管平滑肌细胞中表达的少数几个基因之一。肌动蛋白是标记平滑肌和肌上皮细胞肿瘤的有效工具。

Protein: line1, rat brain   lysates, 30ug; line2, rat kidney lysates, 30ug;

Primary: Anti-alpha-SMA at   1:300;

Secondary: HRP conjugated   Goat-Anti-Rabbit IgG at 1: 3000;

ECL excitated the   fluorescence;

Predicted band size : 42kD

Observed band size : 42kD