{"product_id":"yp-ab-01712-100ul","title":"FKRP Polyclonal Antibody","description":"\u003cul\u003e\n\u003cli\u003e\n\u003cb\u003eGene Name:\u003c\/b\u003e FKRP\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eImmunogen:\u003c\/b\u003e The antiserum was produced against synthesized peptide derived from human FKRP. AA range:1-50\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eSpecificity:\u003c\/b\u003e FKRP Polyclonal Antibody detects endogenous levels of FKRP protein.\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eComposition:\u003c\/b\u003e Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eSource:\u003c\/b\u003e Polyclonal, Rabbit,IgG\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eDilution ratio:\u003c\/b\u003e Western Blot: 1\/500 - 1\/2000. Immunofluorescence: 1\/200 - 1\/1000. ELISA: 1\/20000. Not yet tested in other applications.\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003ePurification process:\u003c\/b\u003e The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eConcentration:\u003c\/b\u003e 1 mg\/ml\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 FKRP; Fukutin-related protein\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eBackground:\u003c\/b\u003e This gene encodes a protein which is targeted to the medial Golgi apparatus and is necessary for posttranslational modification of dystroglycan. Mutations in this gene have been associated with congenital muscular dystrophy, mental retardation, and cerebellar cysts. Several alternatively spliced transcript variants of this gene have been described, but the full-length nature of some of these variants has not been determined. [provided by RefSeq, Oct 2008],\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eFunction:\u003c\/b\u003e disease:Defects in FKRP are the cause of congenital muscular dystrophy type 1C (MDC1C) [MIM:606612]. Congenital muscular dystrophies (CMD) are a heterogeneous group of autosomal recessive disorders characterized by hypotonia, muscle weakness, and joint contractures that present at birth or during the first 6 months of life and have dystrophic changes on skeletal muscle biopsy. Mental retardation with or without structural CNS changes may accompany some forms. MDC1C is a form of CMD with onset in the first weeks of life and a severe phenotype with inability to walk, muscle hypertrophy, marked elevation of serum creatine kinase, a secondary deficiency of laminin alpha2, and a marked reduction in alpha-dystroglycan expression. Only a subset of MDC1C patients have brain involvements.,disease:Defects in FKRP are the cause of limb-girdle muscular dystrophy type 2I (LGMD2I) [MIM:607155]. LGMD2I\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eSpecies:\u003c\/b\u003e Human;Mouse;Rat\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eRange:\u003c\/b\u003e WB;IF;ELISA\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eProtein:\u003c\/b\u003e Fukutin-related protein\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eDA:\u003c\/b\u003e 50kD\u003c\/li\u003e\n\u003c\/ul\u003e","brand":"Upingbio","offers":[{"title":"100UL","offer_id":47780732535008,"sku":"YP-Ab-01712-100UL","price":0.0,"currency_code":"KRW","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0601\/9330\/8896\/files\/images_338cd095-259e-4d3f-9742-33b5b0049b5c.png?v=1758591937","url":"https:\/\/rndmate.com\/products\/yp-ab-01712-100ul","provider":"알앤디메이트","version":"1.0","type":"link"}