Actin (ACTA1) (Muscle Specific) Mouse Monoclonal Antibody [Clone ID: SPM160]

Specifications

Product Data
Clone Name	SPM160
Applications	FC, IF, IHC, IP, WB
Recommend Dilution	Western Blotting:  0.5-1 µg/ml.  Flow Cytometry: 0.5-1 µg/106 cells. Immunoprecipitation: 0.5-1 µg/500 µg protein lysate.  Immunofluorescence: 0.5-1 µg/ml.  Immunohistochemistry on Frozen and Formalin-Fixed Sections: 0.5-1 µg/ml for 30 minutes at RT.  No special pretreatment is required for the immunohistochemical staining of formalin-fixed, paraffin-embedded tissues. Recommended Positive Control: Muscle or sarcoma.
Reactivity	Human, Rabbit, Rat
Host	Mouse
Clonality	Monoclonal
Immunogen	SDS extract of Human myocardium.
Specificity	This antibody recognizes Actin of skeletal, cardiac, and smooth muscle cells. It is not reactive with other mesenchymal cells except for myoepithelium. Actin can be resolved on the basis of its isoelectric points into three distinctive components: alpha, beta, and gamma in order of increasing isoelectric point. Anti-muscle specific actin recognizes alpha and gamma isotype of all muscle groups. Non-muscle cells such as vascular endothelial cells and connective tissues are non-reactive. Also, neoplastic cells of non-muscle-derived tissue such as carcinomas, melanomas, and lymphomas are negative. It stains tumors of smooth muscle (leiomyomas and leiomyosarcomas) as well as skeletal muscle (rhabdomyomas and rhabdomyosarcomas).  Cellular Localization: Cytoplasmic.
Formulation	10mM PBS State: Purified State: Liquid purified IgG fraction from Bioreactor Concentrate Stabilizer: 0.05% BSA Preservative: 0.05% Sodium Azide
Concentration	lot specific
Purification	Protein A/G Chromatography
Conjugation	Unconjugated
Storage Condition	Store undiluted at 2-8°C. DO NOT FREEZE!
Gene Name	actin, alpha 1, skeletal muscle
Database Link	Entrez Gene 58 Human UniProt ID P68133
Background	Function: Actins are highly conserved proteins that are involved in various types of cell motility and are ubiquitously expressed in all eukaryotic cells. Involvement in disease: 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. Sequence similarities: Belongs to the actin family.
Synonyms	ACTA, ACTA1, ACTA2, ACTC1, Actin, ACTSA, Alpha-2 actin, alpha skeletal muscle, Alpha-actin-1, Cardiac muscle alpha actin-1, Skeletal muscle alpha actin-1
Reference Data

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