{"product_id":"yp-ab-02987-10ul","title":"α skeletal muscle actin Monoclonal Antibody(4B11)","description":"\u003cul\u003e\n\u003cli\u003e\n\u003cb\u003eReaction species:\u003c\/b\u003e Human;Mouse;Rat\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eGene Name:\u003c\/b\u003e ACTA1\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eProtein name:\u003c\/b\u003e Actin alpha skeletal muscle\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eMolecular weight (DA):\u003c\/b\u003e 42kD\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eImmunogen:\u003c\/b\u003e Synthetic Peptide of α skeletal muscle actin\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eSpecificity:\u003c\/b\u003e The antibody detects endogenous α Skeletal Muscle Actin protein.\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eComposition:\u003c\/b\u003e PBS, pH 7.4, containing 0.5%BSA, 0.02% sodium azide as Preservative and 50% Glycerol.\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eSource:\u003c\/b\u003e Monoclonal, Mouse\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eDilution ratio:\u003c\/b\u003e WB: 1:500-10000 IP:1:200 IF 1:200 IHC 1:50-300\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003ePurification process:\u003c\/b\u003e The antibody was affinity-purified from mouse ascites by affinity-chromatography using specific immunogen.\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eStorage:\u003c\/b\u003e -20°C\/1 year\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eOther Names:\u003c\/b\u003e ACTA1; ACTA; Actin, alpha skeletal muscle; Alpha-actin-1\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eBackground:\u003c\/b\u003e 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],\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eFunction:\u003c\/b\u003e 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\u003c\/li\u003e\n\u003c\/ul\u003e","brand":"Upingbio","offers":[{"title":"10UL","offer_id":50688875626776,"sku":"YP-Ab-02987-10UL","price":0.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0590\/5652\/1400\/files\/images_603f5b71-683b-47e6-84fb-b202e7bdbe6b.png?v=1758556667","url":"https:\/\/danabiosci.com\/products\/yp-ab-02987-10ul","provider":"Dana Bioscience","version":"1.0","type":"link"}