BD-PT0097

Actin α1 Rabbit Polyclonal Antibody

ACTA1; ACTA; Actin; alpha skeletal muscle; Alpha-actin-1

常规抗体

ACTA1

Actin alpha skeletal muscle

WB

Human,Mouse,Rat

1 mg/ml

Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.

58

http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=58

P68133

http://www.uniprot.org/uniprotkb/P68133/entry

11459

http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=11459

P68134

http://www.uniprot.org/uniprot/P68134

29437

http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=29437

P68136

http://www.uniprot.org/uniprot/P68136

The antiserum was produced against synthesized peptide derived from human Actin-alpha-1. AA range:1-50

Actin α1 Polyclonal Antibody detects endogenous levels of Actin α1 protein.

WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:20000.. IF 1:50-200

45kD

-20°C/1 year

Polyclonal, Rabbit,IgG

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],

Epithelium,Skeletal muscle,

Cytoplasm, cytoskeleton.

Adherens_Junction

disease:Defects in ACTA1 are a cause of congenital myopathy with excess of thin myofilaments (CM) [MIM:102610].,disease: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.,disease:Defects in ACTA1 are the cause of nemaline myopathy type 3 (NEM3) [MIM:161800]. Nemaline myopathy (NEM) is a form of congenital myopathy characterized by abnormal thread- or rod-like structures in muscle fibers on histologic examination. The clinical phenotype is highly variable, with differing age at onset and severity.,function:Actins are highly conserved proteins that are involved in various types of cell motility and are ubiquitously expressed in all eukaryotic cells.,miscellaneous:In vertebrates 3 main groups of actin isoforms, alpha, beta and gamma have been identified. The alpha actins are found in muscle tissues and are a major constituent of the contractile apparatus. The beta and gamma actins coexist in most cell types as components of the cytoskeleton and as mediators of internal cell motility.,similarity:Belongs to the actin family.,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. Interacts with TTID.,

现货

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.