Key features and details
- Rabbit polyclonal to Alpha Skeletal Muscle Actin
- Suitable for: WB
- Reacts with: Human
- Isotype: IgG
- 倫理基準に準拠 - アニマル・フリーの生産
製品名Anti-Alpha Skeletal Muscle Actin antibody
Alpha Skeletal Muscle Actin 一次抗体 製品一覧
製品の詳細Rabbit polyclonal to Alpha Skeletal Muscle Actin
アプリケーション適用あり: WBmore details
交差が予測される動物種: Cow, Zebrafish
Recombinant protein fragment corresponding to a region within amino acids 1 - 129 of Human skeletal muscle Actin (NP_001091).
- MOLT4 and Raji cell lysates
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保存方法Shipped at 4°C. Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
Preservative: 0.025% Proclin 300
Constituents: 79% PBS, 20% Glycerol (glycerin, glycerine)
Concentration information loading...
精製度Immunogen affinity purified
機能Actins are highly conserved proteins that are involved in various types of cell motility and are ubiquitously expressed in all eukaryotic cells.
関連疾患Defects in ACTA1 are the cause of nemaline myopathy type 3 (NEM3) [MIM:161800]. A form of nemaline myopathy. Nemaline myopathies are muscular disorders characterized by muscle weakness of varying severity and onset, and abnormal thread-or rod-like structures in muscle fibers on histologic examination. The phenotype at histological level is variable. Some patients present areas devoid of oxidative activity containg (cores) within myofibers. Core lesions are unstructured and poorly circumscribed.
Defects in ACTA1 are a cause of myopathy congenital with excess of thin myofilaments (MPCETM) [MIM:161800]. A congenital muscular disorder characterized at histological level by areas of sarcoplasm devoid of normal myofibrils and mitochondria, and replaced with dense masses of thin filaments. Central cores, rods, ragged red fibers, and necrosis are absent.
Defects in ACTA1 are a cause of congenital myopathy with fiber-type disproportion (CFTD) [MIM:255310]; also known as congenital fiber-type disproportion myopathy (CFTDM). CFTD is a genetically heterogeneous disorder in which there is relative hypotrophy of type 1 muscle fibers compared to type 2 fibers on skeletal muscle biopsy. However, these findings are not specific and can be found in many different myopathic and neuropathic conditions.
配列類似性Belongs to the actin family.
細胞内局在Cytoplasm > cytoskeleton.
- Information by UniProt
- a actin antibody
- ACTA antibody
- ACTA1 antibody
ab97373 は 1 報の論文で使用されています。
- Anderson BR et al. Allele-Selective Knockdown of MYH7 Using Antisense Oligonucleotides. Mol Ther Nucleic Acids 19:1290-1298 (2020). PubMed: 32092825