Monani UR - Search Results

1

Spectrum of neuropathophysiology in spinal muscular atrophy type I.

Harding BN, Kariya S, Monani UR, Chung WK, Benton M, Yum SW, Tennekoon G, Finkel RS. Harding BN, et al. Among authors: monani ur. J Neuropathol Exp Neurol. 2015 Jan;74(1):15-24. doi: 10.1097/NEN.0000000000000144. J Neuropathol Exp Neurol. 2015. PMID: 25470343 Free PMC article.

2

Spinal muscular atrophy: a deficiency in a ubiquitous protein; a motor neuron-specific disease.

Monani UR. Monani UR. Neuron. 2005 Dec 22;48(6):885-96. doi: 10.1016/j.neuron.2005.12.001. Neuron. 2005. PMID: 16364894 Free article. Review.

3

Reduced SMN protein impairs maturation of the neuromuscular junctions in mouse models of spinal muscular atrophy.

Kariya S, Park GH, Maeno-Hikichi Y, Leykekhman O, Lutz C, Arkovitz MS, Landmesser LT, Monani UR. Kariya S, et al. Among authors: monani ur. Hum Mol Genet. 2008 Aug 15;17(16):2552-69. doi: 10.1093/hmg/ddn156. Epub 2008 May 20. Hum Mol Genet. 2008. PMID: 18492800 Free PMC article.

4

The neuroprotective factor Wld(s) fails to mitigate distal axonal and neuromuscular junction (NMJ) defects in mouse models of spinal muscular atrophy.

Kariya S, Mauricio R, Dai Y, Monani UR. Kariya S, et al. Among authors: monani ur. Neurosci Lett. 2009 Jan 16;449(3):246-51. doi: 10.1016/j.neulet.2008.10.107. Epub 2008 Nov 8. Neurosci Lett. 2009. PMID: 19010394 Free PMC article.

5

Spinal muscular atrophy: new and emerging insights from model mice.

Park GH, Kariya S, Monani UR. Park GH, et al. Among authors: monani ur. Curr Neurol Neurosci Rep. 2010 Mar;10(2):108-17. doi: 10.1007/s11910-010-0095-5. Curr Neurol Neurosci Rep. 2010. PMID: 20425235 Free PMC article. Review.

6

Reduced survival of motor neuron (SMN) protein in motor neuronal progenitors functions cell autonomously to cause spinal muscular atrophy in model mice expressing the human centromeric (SMN2) gene.

Park GH, Maeno-Hikichi Y, Awano T, Landmesser LT, Monani UR. Park GH, et al. Among authors: monani ur. J Neurosci. 2010 Sep 8;30(36):12005-19. doi: 10.1523/JNEUROSCI.2208-10.2010. J Neurosci. 2010. PMID: 20826664 Free PMC article.

7

Postsymptomatic restoration of SMN rescues the disease phenotype in a mouse model of severe spinal muscular atrophy.

Lutz CM, Kariya S, Patruni S, Osborne MA, Liu D, Henderson CE, Li DK, Pellizzoni L, Rojas J, Valenzuela DM, Murphy AJ, Winberg ML, Monani UR. Lutz CM, et al. Among authors: monani ur. J Clin Invest. 2011 Aug;121(8):3029-41. doi: 10.1172/JCI57291. Epub 2011 Jul 25. J Clin Invest. 2011. PMID: 21785219 Free PMC article.

8

A role for SMN exon 7 splicing in the selective vulnerability of motor neurons in spinal muscular atrophy.

Ruggiu M, McGovern VL, Lotti F, Saieva L, Li DK, Kariya S, Monani UR, Burghes AH, Pellizzoni L. Ruggiu M, et al. Among authors: monani ur. Mol Cell Biol. 2012 Jan;32(1):126-38. doi: 10.1128/MCB.06077-11. Epub 2011 Oct 28. Mol Cell Biol. 2012. PMID: 22037760 Free PMC article.

9

Mutant superoxide dismutase 1 (SOD1), a cause of amyotrophic lateral sclerosis, disrupts the recruitment of SMN, the spinal muscular atrophy protein to nuclear Cajal bodies.

Kariya S, Re DB, Jacquier A, Nelson K, Przedborski S, Monani UR. Kariya S, et al. Among authors: monani ur. Hum Mol Genet. 2012 Aug 1;21(15):3421-34. doi: 10.1093/hmg/dds174. Epub 2012 May 11. Hum Mol Genet. 2012. PMID: 22581780 Free PMC article.

10

Limited phenotypic effects of selectively augmenting the SMN protein in the neurons of a mouse model of severe spinal muscular atrophy.

Lee AJ, Awano T, Park GH, Monani UR. Lee AJ, et al. Among authors: monani ur. PLoS One. 2012;7(9):e46353. doi: 10.1371/journal.pone.0046353. Epub 2012 Sep 27. PLoS One. 2012. PMID: 23029491 Free PMC article.

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