Cargando…
Loss of Zebrafish lgi1b Leads to Hydrocephalus and Sensitization to Pentylenetetrazol Induced Seizure-Like Behavior
Mutations in the LGI1 gene predispose to a hereditary epilepsy syndrome and is the first gene associated with this disease which does not encode an ion channel protein. In zebrafish, there are two paralogs of the LGI1 gene, lgi1a and lgi1b. Knockdown of lgi1a results in a seizure-like hyperactivity...
Autores principales: | , , , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2011
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3203530/ https://www.ncbi.nlm.nih.gov/pubmed/22053218 http://dx.doi.org/10.1371/journal.pone.0024596 |
_version_ | 1782215118840397824 |
---|---|
author | Teng, Yong Xie, Xiayang Walker, Steven Saxena, Meera Kozlowski, David J. Mumm, Jeff S. Cowell, John K. |
author_facet | Teng, Yong Xie, Xiayang Walker, Steven Saxena, Meera Kozlowski, David J. Mumm, Jeff S. Cowell, John K. |
author_sort | Teng, Yong |
collection | PubMed |
description | Mutations in the LGI1 gene predispose to a hereditary epilepsy syndrome and is the first gene associated with this disease which does not encode an ion channel protein. In zebrafish, there are two paralogs of the LGI1 gene, lgi1a and lgi1b. Knockdown of lgi1a results in a seizure-like hyperactivity phenotype with associated developmental abnormalities characterized by cellular loss in the eyes and brain. We have now generated knockdown morphants for the lgi1b gene which also show developmental abnormalities but do not show a seizure-like behavior. Instead, the most striking phenotype involves significant enlargement of the ventricles (hydrocephalus). As shown for the lgi1a morphants, however, lgi1b morphants are also sensitized to PTZ-induced hyperactivity. The different phenotypes between the two lgi1 morphants support a subfunctionalization model for the two paralogs. |
format | Online Article Text |
id | pubmed-3203530 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2011 |
publisher | Public Library of Science |
record_format | MEDLINE/PubMed |
spelling | pubmed-32035302011-11-03 Loss of Zebrafish lgi1b Leads to Hydrocephalus and Sensitization to Pentylenetetrazol Induced Seizure-Like Behavior Teng, Yong Xie, Xiayang Walker, Steven Saxena, Meera Kozlowski, David J. Mumm, Jeff S. Cowell, John K. PLoS One Research Article Mutations in the LGI1 gene predispose to a hereditary epilepsy syndrome and is the first gene associated with this disease which does not encode an ion channel protein. In zebrafish, there are two paralogs of the LGI1 gene, lgi1a and lgi1b. Knockdown of lgi1a results in a seizure-like hyperactivity phenotype with associated developmental abnormalities characterized by cellular loss in the eyes and brain. We have now generated knockdown morphants for the lgi1b gene which also show developmental abnormalities but do not show a seizure-like behavior. Instead, the most striking phenotype involves significant enlargement of the ventricles (hydrocephalus). As shown for the lgi1a morphants, however, lgi1b morphants are also sensitized to PTZ-induced hyperactivity. The different phenotypes between the two lgi1 morphants support a subfunctionalization model for the two paralogs. Public Library of Science 2011-09-16 /pmc/articles/PMC3203530/ /pubmed/22053218 http://dx.doi.org/10.1371/journal.pone.0024596 Text en Teng et al. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are properly credited. |
spellingShingle | Research Article Teng, Yong Xie, Xiayang Walker, Steven Saxena, Meera Kozlowski, David J. Mumm, Jeff S. Cowell, John K. Loss of Zebrafish lgi1b Leads to Hydrocephalus and Sensitization to Pentylenetetrazol Induced Seizure-Like Behavior |
title | Loss of Zebrafish lgi1b Leads to Hydrocephalus and Sensitization to Pentylenetetrazol Induced Seizure-Like Behavior |
title_full | Loss of Zebrafish lgi1b Leads to Hydrocephalus and Sensitization to Pentylenetetrazol Induced Seizure-Like Behavior |
title_fullStr | Loss of Zebrafish lgi1b Leads to Hydrocephalus and Sensitization to Pentylenetetrazol Induced Seizure-Like Behavior |
title_full_unstemmed | Loss of Zebrafish lgi1b Leads to Hydrocephalus and Sensitization to Pentylenetetrazol Induced Seizure-Like Behavior |
title_short | Loss of Zebrafish lgi1b Leads to Hydrocephalus and Sensitization to Pentylenetetrazol Induced Seizure-Like Behavior |
title_sort | loss of zebrafish lgi1b leads to hydrocephalus and sensitization to pentylenetetrazol induced seizure-like behavior |
topic | Research Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3203530/ https://www.ncbi.nlm.nih.gov/pubmed/22053218 http://dx.doi.org/10.1371/journal.pone.0024596 |
work_keys_str_mv | AT tengyong lossofzebrafishlgi1bleadstohydrocephalusandsensitizationtopentylenetetrazolinducedseizurelikebehavior AT xiexiayang lossofzebrafishlgi1bleadstohydrocephalusandsensitizationtopentylenetetrazolinducedseizurelikebehavior AT walkersteven lossofzebrafishlgi1bleadstohydrocephalusandsensitizationtopentylenetetrazolinducedseizurelikebehavior AT saxenameera lossofzebrafishlgi1bleadstohydrocephalusandsensitizationtopentylenetetrazolinducedseizurelikebehavior AT kozlowskidavidj lossofzebrafishlgi1bleadstohydrocephalusandsensitizationtopentylenetetrazolinducedseizurelikebehavior AT mummjeffs lossofzebrafishlgi1bleadstohydrocephalusandsensitizationtopentylenetetrazolinducedseizurelikebehavior AT cowelljohnk lossofzebrafishlgi1bleadstohydrocephalusandsensitizationtopentylenetetrazolinducedseizurelikebehavior |