Argininosuccinic aciduria fosters neuronal nitrosative stress reversed by Asl gene transfer
Patients with mutations in the ASL gene present with argininosuccinic aciduria characterised by hyperammonaemia and cognitive impairment. Here, the authors show that cerebral disease involves neuronal nitrosative/oxidative stress that is not induced by hyperammonaemia, and that it can be reversed us...
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Nature Portfolio
2018
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oai:doaj.org-article:bccd48b998f7466b8b4e37f6409acdb22021-12-02T14:39:45ZArgininosuccinic aciduria fosters neuronal nitrosative stress reversed by Asl gene transfer10.1038/s41467-018-05972-12041-1723https://doaj.org/article/bccd48b998f7466b8b4e37f6409acdb22018-08-01T00:00:00Zhttps://doi.org/10.1038/s41467-018-05972-1https://doaj.org/toc/2041-1723Patients with mutations in the ASL gene present with argininosuccinic aciduria characterised by hyperammonaemia and cognitive impairment. Here, the authors show that cerebral disease involves neuronal nitrosative/oxidative stress that is not induced by hyperammonaemia, and that it can be reversed using AAV-ASL directed to liver and brain in mice.Julien BaruteauDany P. PerocheauJoanna HanleyMaëlle LorvellecEridan Rocha-FerreiraRajvinder KardaJoanne NgNatalie SuffJuan Antinao DiazAhad A. RahimMichael P. HughesBlerida BanushiHelen PruntyMariya HristovaDeborah A. RidoutAlex VirasamiSimon HealesStewen J. HoweSuzanne M. K. BuckleyPhilippa B. MillsPaul GissenSimon N. WaddingtonNature PortfolioarticleScienceQENNature Communications, Vol 9, Iss 1, Pp 1-14 (2018) |
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DOAJ |
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EN |
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Science Q |
| spellingShingle |
Science Q Julien Baruteau Dany P. Perocheau Joanna Hanley Maëlle Lorvellec Eridan Rocha-Ferreira Rajvinder Karda Joanne Ng Natalie Suff Juan Antinao Diaz Ahad A. Rahim Michael P. Hughes Blerida Banushi Helen Prunty Mariya Hristova Deborah A. Ridout Alex Virasami Simon Heales Stewen J. Howe Suzanne M. K. Buckley Philippa B. Mills Paul Gissen Simon N. Waddington Argininosuccinic aciduria fosters neuronal nitrosative stress reversed by Asl gene transfer |
| description |
Patients with mutations in the ASL gene present with argininosuccinic aciduria characterised by hyperammonaemia and cognitive impairment. Here, the authors show that cerebral disease involves neuronal nitrosative/oxidative stress that is not induced by hyperammonaemia, and that it can be reversed using AAV-ASL directed to liver and brain in mice. |
| format |
article |
| author |
Julien Baruteau Dany P. Perocheau Joanna Hanley Maëlle Lorvellec Eridan Rocha-Ferreira Rajvinder Karda Joanne Ng Natalie Suff Juan Antinao Diaz Ahad A. Rahim Michael P. Hughes Blerida Banushi Helen Prunty Mariya Hristova Deborah A. Ridout Alex Virasami Simon Heales Stewen J. Howe Suzanne M. K. Buckley Philippa B. Mills Paul Gissen Simon N. Waddington |
| author_facet |
Julien Baruteau Dany P. Perocheau Joanna Hanley Maëlle Lorvellec Eridan Rocha-Ferreira Rajvinder Karda Joanne Ng Natalie Suff Juan Antinao Diaz Ahad A. Rahim Michael P. Hughes Blerida Banushi Helen Prunty Mariya Hristova Deborah A. Ridout Alex Virasami Simon Heales Stewen J. Howe Suzanne M. K. Buckley Philippa B. Mills Paul Gissen Simon N. Waddington |
| author_sort |
Julien Baruteau |
| title |
Argininosuccinic aciduria fosters neuronal nitrosative stress reversed by Asl gene transfer |
| title_short |
Argininosuccinic aciduria fosters neuronal nitrosative stress reversed by Asl gene transfer |
| title_full |
Argininosuccinic aciduria fosters neuronal nitrosative stress reversed by Asl gene transfer |
| title_fullStr |
Argininosuccinic aciduria fosters neuronal nitrosative stress reversed by Asl gene transfer |
| title_full_unstemmed |
Argininosuccinic aciduria fosters neuronal nitrosative stress reversed by Asl gene transfer |
| title_sort |
argininosuccinic aciduria fosters neuronal nitrosative stress reversed by asl gene transfer |
| publisher |
Nature Portfolio |
| publishDate |
2018 |
| url |
https://doaj.org/article/bccd48b998f7466b8b4e37f6409acdb2 |
| work_keys_str_mv |
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