{"product_id":"new-product-225488","title":"MYOT polyclonal antibody","description":"\u003cp\u003eMyotilin, a sarcomeric protein that is encoded by the gene mapping to human chromosome 5q31, binds to a-actinin and is localized in the Z-line of myofibrils . Myotilin is expressed in skeletal and cardiac muscle, and it co-localizes with a-actinin in the sarcomeric I-bands where it directly interacts with a-actinin . Defects in the myotilin gene are reported to cause a form of autosomal dominant limb-girdle muscular dystrophy (LGMD) (1). Symptoms of adult onset LGMD are progressive weakness of the hip and shoulder girdles as well as a distinctive dysarthric pattern of speech . The muscle of affected individuals with LGMD shows degeneration of myofibers, variations in fiber size, fiber splitting, centrally located myonuclei and an enhanced number of autophagic vesicles .\u003c\/p\u003e","brand":"Bioworld","offers":[{"title":"100ul \/ Rabbit \/ Human,Mouse,Rat","offer_id":47661863207192,"sku":"BS8217","price":408.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0590\/5652\/1400\/products\/BIOWORLD_58ea4937-71cd-4e9c-8f0c-8080da9d806b.jpg?v=1707469498","url":"https:\/\/danabiosci.com\/products\/new-product-225488","provider":"Dana Bioscience","version":"1.0","type":"link"}