Antigen Preparation
"A synthetic peptide corresponding to the C-terminus of human FKRP protein. This sequence is identical to human, mouse and rat"
Background
"Fukutin-related protein (FKRP) 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. Mutations in fukutin-related protein (FKRP) give rise to mild and more severe forms of muscular dystrophy. FKRP may influence the glycosylation status of dystroglycan from within the sarcolemmal dystrophin-glycoprotein complex. FKRP associates with the sarcolemmal DGC and that it plays a unique role in dystroglycanopathy muscle disease."
Applications/Suggested Working Dilutions
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Immunoprecipitation
2-5 µg/ml
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Flow cytometry
Not tested
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