U-Blot® Alpha skeletal muscle Actin Rabbit mAb
Ucallm_WR5908-50
Price range: $268.00 through $328.00
Product details
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] ,
Specifications
| Size | 50μL/100μL |
| Target | Alpha skeletal muscle Actin |
| Reactivity | Human, Mouse, Rat |
| Applications | WB, IHC, IF, IP, ELISA |
| MW(Calculated) | 42kD |
| MW(Observed) | 42kD |
| Host Species | Rabbit |
| Isotype | IgG,Kappa |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | WB 1:500-1:2000; IF 1:200-1:1000; ELISA 1:5000-1:20000; IP 1:50-1:200; |
| Formulation | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| Purification | Recombinant Antibody expressed in animal component-free (ACF) media, purified via Protein A affinity chromatography. |
| Storage | -15°C to -25°C/1 year(Do not lower than -25°C) |
| Concentration | |
| Clonality | Monoclonal |
| Clone Number | RM1910R |
| Immunogen | The specific immunogen used to produce this antibody is proprietary information. |
| Specificity | Endogenous |
| Gene Name | ACTA1 |
| Protein Name | Alpha skeletal muscle Actin |
| Other Name | ACTA1; / ACTA; / Actin; / alpha skeletal muscle; / Alpha-actin-1 |
| Species | Human |
| Gene ID | 58 |
| Uniprot | P68133, |
| Species.1 | Mouse |
| Gene ID.1 | 11459 |
| Uniprot.1 | P68134, |
| Species.2 | Rat |
| Gene ID.2 | 29437 |
| Uniprot.2 | P68136 |
| Cellular Localization | Cytoplasm, cytoskeleton. |
| Tissue Expression | Epithelium,Skeletal muscle, |
| Signaling_pathway | |
| Research Areas | |
| 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.,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., |
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