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- W2144865179 endingPage "2059" @default.
- W2144865179 startingPage "2043" @default.
- W2144865179 abstract "Cortical malformations are a collection of disorders affecting brain development. Mutations in the LIS1 gene lead to a disorganized and smooth cerebral cortex caused by failure in neuronal migration. Among the clinical consequences of lissencephaly are mental retardation and intractable epilepsy. It remains unclear whether the seizures result from aberrant neuronal placement, disruption of intrinsic properties of neurons, or both. The nematode Caenorhabditis elegans offers an opportunity to study such convulsions in a simple animal with a defined nervous system. Here we show that convulsions mimicking epilepsy can be induced by a mutation in a C. elegans lis-1 allele ( pnm-1 ), in combination with a chemical antagonist of gamma-aminobutyric acid (GABA) neurotransmitter signaling. Identical convulsions were obtained using C. elegans mutants defective in GABA transmission, whereas none of these mutants or the antagonist alone caused convulsions, indicating a threshold was exceeded in response to this combination. Crosses between pnm-1 and fluorescent marker strains designed to exclusively illuminate either the processes of GABAergic neurons or synaptic vesicles surprisingly showed no deviations in neuronal architecture. Instead, presynaptic defects in GABAergic vesicle distribution were clearly evident and could be phenocopied by RNAi directed against cytoplasmic dynein, a known LIS1 interactor. Furthermore, mutations in UNC-104, a neuronal-specific kinesin, and SNB-1, a synaptic vesicle-associated protein termed synaptobrevin, exhibit similar convulsion phenotypes following chemical induction. Taken together, these studies establish C. elegans as a system to investigate subtle cytoskeletal mechanisms regulating intrinsic neuronal activity and suggest that it may be possible to dissociate the epileptic consequences of lissencephaly from the more phenotypically overt cortical defects associated with neuronal migration." @default.
- W2144865179 created "2016-06-24" @default.
- W2144865179 creator A5019656708 @default.
- W2144865179 creator A5033267154 @default.
- W2144865179 creator A5039512622 @default.
- W2144865179 creator A5060179135 @default.
- W2144865179 date "2004-07-14" @default.
- W2144865179 modified "2023-10-05" @default.
- W2144865179 title "Epileptic-like convulsions associated with LIS-1 in the cytoskeletal control of neurotransmitter signaling in Caenorhabditis elegans" @default.
- W2144865179 cites W1481912803 @default.
- W2144865179 cites W1484928190 @default.
- W2144865179 cites W1490197708 @default.
- W2144865179 cites W1495907309 @default.
- W2144865179 cites W1505732106 @default.
- W2144865179 cites W1532388773 @default.
- W2144865179 cites W1539795260 @default.
- W2144865179 cites W1561680043 @default.
- W2144865179 cites W1580418835 @default.
- W2144865179 cites W1630027754 @default.
- W2144865179 cites W1944127002 @default.
- W2144865179 cites W1944684909 @default.
- W2144865179 cites W1966420577 @default.
- W2144865179 cites W1967781608 @default.
- W2144865179 cites W1968193711 @default.
- W2144865179 cites W1970182993 @default.
- W2144865179 cites W1970689148 @default.
- W2144865179 cites W1971126847 @default.
- W2144865179 cites W1971447039 @default.
- W2144865179 cites W1972207175 @default.
- W2144865179 cites W1973160416 @default.
- W2144865179 cites W1984979116 @default.
- W2144865179 cites W1992502425 @default.
- W2144865179 cites W1997564419 @default.
- W2144865179 cites W2001275760 @default.
- W2144865179 cites W2001281440 @default.
- W2144865179 cites W2007499634 @default.
- W2144865179 cites W2012687647 @default.
- W2144865179 cites W2012809099 @default.
- W2144865179 cites W2012855723 @default.
- W2144865179 cites W2021295015 @default.
- W2144865179 cites W2029120000 @default.
- W2144865179 cites W2029641279 @default.
- W2144865179 cites W2036623899 @default.
- W2144865179 cites W2037835429 @default.
- W2144865179 cites W2047454656 @default.
- W2144865179 cites W2049469649 @default.
- W2144865179 cites W2052403810 @default.
- W2144865179 cites W2053775289 @default.
- W2144865179 cites W2055543039 @default.
- W2144865179 cites W2057952525 @default.
- W2144865179 cites W2058219169 @default.
- W2144865179 cites W2061300314 @default.
- W2144865179 cites W2063096457 @default.
- W2144865179 cites W2066713988 @default.
- W2144865179 cites W2067340269 @default.
- W2144865179 cites W2067890396 @default.
- W2144865179 cites W2068805332 @default.
- W2144865179 cites W2072596507 @default.
- W2144865179 cites W2073514506 @default.
- W2144865179 cites W2074888950 @default.
- W2144865179 cites W2077347535 @default.
- W2144865179 cites W2079441999 @default.
- W2144865179 cites W2080579127 @default.
- W2144865179 cites W2089500651 @default.
- W2144865179 cites W2092920672 @default.
- W2144865179 cites W2095790288 @default.
- W2144865179 cites W2097433384 @default.
- W2144865179 cites W2098876907 @default.
- W2144865179 cites W2098877593 @default.
- W2144865179 cites W2102875752 @default.
- W2144865179 cites W2104529374 @default.
- W2144865179 cites W2105851524 @default.
- W2144865179 cites W2106790290 @default.
- W2144865179 cites W2108796191 @default.
- W2144865179 cites W2109975756 @default.
- W2144865179 cites W2110029962 @default.
- W2144865179 cites W2115047272 @default.
- W2144865179 cites W2116811981 @default.
- W2144865179 cites W2123607569 @default.
- W2144865179 cites W2125625974 @default.
- W2144865179 cites W2127012355 @default.
- W2144865179 cites W2128647989 @default.
- W2144865179 cites W2128804867 @default.
- W2144865179 cites W2134505334 @default.
- W2144865179 cites W2135239355 @default.
- W2144865179 cites W2135241130 @default.
- W2144865179 cites W2137976898 @default.
- W2144865179 cites W2140834763 @default.
- W2144865179 cites W2142305917 @default.
- W2144865179 cites W2143255074 @default.
- W2144865179 cites W2144151547 @default.
- W2144865179 cites W2144359192 @default.
- W2144865179 cites W2146515362 @default.
- W2144865179 cites W2146998104 @default.
- W2144865179 cites W2147556035 @default.
- W2144865179 cites W2149871164 @default.
- W2144865179 cites W2155011915 @default.
- W2144865179 cites W2160938187 @default.