Name: U-Blot® Actin, sarcomeric muscle Rabbit mAb (Ready to Use)
SKU: WR8001
Price: 168 USD
Availability: InStock

U-Blot® Actin, sarcomeric muscle Rabbit mAb (Ready to Use)

Ucallm_WR8001

$168.00

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	6mL
Target	Actin_sarcomeric muscle
Reactivity	Human, Mouse, Rat, Bovine, Pig, Chicken
Applications	IHC
MW(Calculated)
MW(Observed)
Host Species	Rabbit
Isotype	IgG1,Kappa
Conjugate/Modification	Unmodified
Recommended Dilution Ratio	Ready to use for IHC / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004)
Formulation	The prediluted ready-to-use antibody is diluted in phosphate buffer saline containing stabilizing protein and 0.05% Proclin 300
Purification	Recombinant Expression and Affinity purified
Storage	2°C to 8°C/1 year,Ship by ice bag
Concentration
Clonality	Monoclonal
Clone Number	RM55R
Immunogen	Synthesized peptide derived from human Actin, sarcomeric muscle AA range:2-50
Specificity	This antibody detects endogenous levels of Actin, sarcomeric muscle
Gene Name	ACTA1; ACTA; ACTC1; ACTC
Protein Name	Actin, sarcomeric muscle
Other Name	Actin, alpha skeletal muscle; / Alpha-actin-1; / ACTA1; / Actin, alpha cardiac muscle 1; / Alpha-cardiac actin; / ACTC1
Species	Human
Gene ID	375;377
Uniprot	P68032
Species.1
Gene ID.1
Uniprot.1
Species.2
Gene ID.2
Uniprot.2
Cellular Localization	Cytoplasm, cytoskeleton.
Tissue Expression	Epithelium,Skeletal muscle,
Signaling_pathway	Environmental Information Processing >> Signal transduction >> Phospholipase D 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.,