Article

Argininosuccinic aciduria fosters neuronal nitrosative stress reversed by <i>Asl</i> gene transfer

2018-06-15

Abstract excerpt

<h4>ABSTRACT</h4> Argininosuccinate lyase (ASL) belongs to the liver-based urea cycle detoxifying ammonia, and the citrulline-nitric oxide cycle synthesising nitric oxide (NO). ASL-deficient patients present argininosuccinic aciduria characterised by hyperammonaemia and a multi-organ disease with neurocognitive impairment. Current therapeutic guidelines aim to control ammonaemia without considering the systemic N...

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Literature Corpus work: 0d310253-f6d2-5edd-9df8-2acdf200f689
DOI: 10.1101/348292

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