Molecular weight (DA)
42kD
Immunogen
Synthesized peptide derived from the C-terminal region of human α-SMA.
Specificity
α-SMA Polyclonal Antibody detects endogenous levels of α-SMA protein.
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
WB: 1/500 - 1/2000. IHC-p: 1:100-300 ELISA: 1/20000.. IF 1:50-200
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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
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.,func
Gene Name
ACTA1/ACTA2/ACTC1
Protein name
Actin alpha skeletal muscle/Actin aortic smooth muscle/Actin alpha cardiac muscle 1
Abbreviation
Actin skeletal muscle α
Other name
ACTA1; ACTA; Actin, alpha skeletal muscle; Alpha-actin-1; ACTA2; ACTSA; ACTVS; GIG46; Actin, aortic smooth muscle; Alpha-actin-2; Cell growth-inhibiting gene 46 protein; ACTC1; ACTC; Actin, alpha cardiac muscle 1; Alpha-cardiac actinACTA1; ACTA; Actin, alpha skeletal muscle; Alpha-actin-1; ACTA2; ACTSA; ACTVS; GIG46; Actin, aortic smooth muscle; Alpha-actin-2; Cell growth-inhibiting gene 46 protein; ACTC1; ACTC; Actin, alpha cardiac muscle 1; Alpha-cardiac actin
Human protein sequence Database
P68133
Mouse protein sequence database
P68134
Rat protein sequence database
P68136
Cellular localization
Cytoplasm, cytoskeleton.
Tissue expression
Epithelium,Skeletal muscle,
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