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- W2133784254 abstract "Proximal spinal muscular atrophy (SMA) is the most frequent cause of hereditary infant mortality. SMA is an autosomal recessive neuromuscular disorder that results from the loss of the Survival Motor Neuron 1 (SMN1) gene and retention of the SMN2 gene. The SMN2 gene produces an insufficient amount of full-length SMN protein that results in loss of motor neurons in the spinal cord and subsequent muscle paralysis. Previously we have shown that overexpression of human SMN in neurons in the SMA mouse ameliorates the SMA phenotype while overexpression of human SMN in skeletal muscle had no effect. Using Cre recombinase, here we show that either deletion or replacement of Smn in motor neurons (ChAT-Cre) significantly alters the functional output of the motor unit as measured with compound muscle action potential and motor unit number estimation. However ChAT-Cre alone did not alter the survival of SMA mice by replacement and did not appreciably affect survival when used to deplete SMN. However replacement of Smn in both neurons and glia in addition to the motor neuron (Nestin-Cre and ChAT-Cre) resulted in the greatest improvement in survival of the mouse and in some instances complete rescue was achieved. These findings demonstrate that high expression of SMN in the motor neuron is both necessary and sufficient for proper function of the motor unit. Furthermore, in the mouse high expression of SMN in neurons and glia, in addition to motor neurons, has a major impact on survival." @default.
- W2133784254 created "2016-06-24" @default.
- W2133784254 creator A5016640472 @default.
- W2133784254 creator A5033396587 @default.
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- W2133784254 creator A5066106247 @default.
- W2133784254 creator A5070681823 @default.
- W2133784254 creator A5070793028 @default.
- W2133784254 date "2015-07-23" @default.
- W2133784254 modified "2023-10-15" @default.
- W2133784254 title "SMN expression is required in motor neurons to rescue electrophysiological deficits in the SMNΔ7 mouse model of SMA" @default.
- W2133784254 cites W120166734 @default.
- W2133784254 cites W1554872850 @default.
- W2133784254 cites W1580690721 @default.
- W2133784254 cites W1757206974 @default.
- W2133784254 cites W1761323037 @default.
- W2133784254 cites W1876029662 @default.
- W2133784254 cites W1893031143 @default.
- W2133784254 cites W1912693339 @default.
- W2133784254 cites W1967242914 @default.
- W2133784254 cites W1969973773 @default.
- W2133784254 cites W1975560789 @default.
- W2133784254 cites W1976199077 @default.
- W2133784254 cites W1977045553 @default.
- W2133784254 cites W1980477780 @default.
- W2133784254 cites W1983600034 @default.
- W2133784254 cites W1985468175 @default.
- W2133784254 cites W1986164507 @default.
- W2133784254 cites W1986864832 @default.
- W2133784254 cites W1987687980 @default.
- W2133784254 cites W1992985034 @default.
- W2133784254 cites W1996417439 @default.
- W2133784254 cites W1996670527 @default.
- W2133784254 cites W1998552977 @default.
- W2133784254 cites W1999848446 @default.
- W2133784254 cites W2000101032 @default.
- W2133784254 cites W2000725203 @default.
- W2133784254 cites W2002912370 @default.
- W2133784254 cites W2005873273 @default.
- W2133784254 cites W2009914881 @default.
- W2133784254 cites W2015026135 @default.
- W2133784254 cites W2015574237 @default.
- W2133784254 cites W2016855436 @default.
- W2133784254 cites W2018217165 @default.
- W2133784254 cites W2019638822 @default.
- W2133784254 cites W2025722577 @default.
- W2133784254 cites W2027120092 @default.
- W2133784254 cites W2032742781 @default.
- W2133784254 cites W2034784666 @default.
- W2133784254 cites W2036591210 @default.
- W2133784254 cites W2040574649 @default.
- W2133784254 cites W2042400521 @default.
- W2133784254 cites W2045117117 @default.
- W2133784254 cites W2046533709 @default.
- W2133784254 cites W2050034509 @default.
- W2133784254 cites W2052064222 @default.
- W2133784254 cites W2052528763 @default.
- W2133784254 cites W2054236204 @default.
- W2133784254 cites W2054380003 @default.
- W2133784254 cites W2059920245 @default.
- W2133784254 cites W2068346771 @default.
- W2133784254 cites W2070007143 @default.
- W2133784254 cites W2070086105 @default.
- W2133784254 cites W2074147019 @default.
- W2133784254 cites W2081384639 @default.
- W2133784254 cites W2085465310 @default.
- W2133784254 cites W2088151363 @default.
- W2133784254 cites W2095511833 @default.
- W2133784254 cites W2099624163 @default.
- W2133784254 cites W2100163011 @default.
- W2133784254 cites W2101970831 @default.
- W2133784254 cites W2104135721 @default.
- W2133784254 cites W2105989886 @default.
- W2133784254 cites W2107257806 @default.
- W2133784254 cites W2112430789 @default.
- W2133784254 cites W2115034448 @default.
- W2133784254 cites W2115222513 @default.
- W2133784254 cites W2115575892 @default.
- W2133784254 cites W2116175665 @default.
- W2133784254 cites W2117278447 @default.
- W2133784254 cites W2118748690 @default.
- W2133784254 cites W2119330162 @default.
- W2133784254 cites W2119878613 @default.
- W2133784254 cites W2121171425 @default.
- W2133784254 cites W2125953041 @default.
- W2133784254 cites W2129294012 @default.
- W2133784254 cites W2129447667 @default.
- W2133784254 cites W2132519155 @default.
- W2133784254 cites W2136868631 @default.
- W2133784254 cites W2137021233 @default.
- W2133784254 cites W2138161663 @default.
- W2133784254 cites W2140853061 @default.
- W2133784254 cites W2142120640 @default.
- W2133784254 cites W2142845441 @default.
- W2133784254 cites W2143930757 @default.
- W2133784254 cites W2147552101 @default.