Utrophin upregulation for treating Duchenne or Becker muscular dystrophy: how close are we?

作者: Pedro Miura , Bernard J. Jasmin

DOI: 10.1016/J.MOLMED.2006.01.002

关键词: Regulation of gene expressionDownregulation and upregulationBioinformaticsUtrophinDystrophinSarcolemmaMedicineDuchenne muscular dystrophyNeuregulin 1Muscular dystrophy

摘要: … RhoA has been implicated in activating utrophin transcription … into effective long-term pharmacotherapeutics for DMD patients (… contribute to maintaining high levels of utrophin …

参考文章(82)
L Politano, S Papparella, L I Comi, V Nigro, O Paciello, G Piluso, G Nigro, Gentamicin administration in Duchenne patients with premature stop codon. Preliminary results. Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology / edited by the Gaetano Conte Academy for the study of striated muscle diseases. ,vol. 22, pp. 15- 21 ,(2003)
Elisabeth R. Barton, Linda Morris, Masataka Kawana, Lawrence T. Bish, Thierry Toursel, Systemic administration of L-arginine benefits mdx skeletal muscle function. Muscle & Nerve. ,vol. 32, pp. 751- 760 ,(2005) , 10.1002/MUS.20425
V VOISIN, C SEBRIE, S MATECKI, H YU, B GILLET, M RAMONATXO, M ISRAEL, S DELAPORTE, l-arginine improves dystrophic phenotype in mdx mice Neurobiology of Disease. ,vol. 20, pp. 123- 130 ,(2005) , 10.1016/J.NBD.2005.02.010
Jonathan S. Stamler, Gerhard Meissner, Physiology of Nitric Oxide in Skeletal Muscle Physiological Reviews. ,vol. 81, pp. 209- 237 ,(2001) , 10.1152/PHYSREV.2001.81.1.209
Shuo Lin, Jean-Marc Burgunder, Utrophin may be a precursor of dystrophin during skeletal muscle development. Developmental Brain Research. ,vol. 119, pp. 289- 295 ,(2000) , 10.1016/S0165-3806(99)00165-0
Paul Gregorevic, Michael J Blankinship, James M Allen, Robert W Crawford, Leonard Meuse, Daniel G Miller, David W Russell, Jeffrey S Chamberlain, Systemic delivery of genes to striated muscles using adeno-associated viral vectors. Nature Medicine. ,vol. 10, pp. 828- 834 ,(2004) , 10.1038/NM1085
Kelly J Perkins, Kay E Davies, The role of utrophin in the potential therapy of Duchenne muscular dystrophy Neuromuscular Disorders. ,vol. 12, ,(2002) , 10.1016/S0960-8966(02)00087-1
Raghavan Madhavan, Larry R. Massom, Harry W. Jarrett, Calmodulin specifically binds three proteins of the dystrophin-glycoprotein complex Biochemical and Biophysical Research Communications. ,vol. 185, pp. 753- 759 ,(1992) , 10.1016/0006-291X(92)91690-R