Original paper

Argininosuccinic aciduria fosters neuronal nitrosative stress reversed by Asl gene transfer

Volume: 9, Issue: 1
Published: Aug 29, 2018
Abstract
Argininosuccinate lyase (ASL) belongs to the hepatic urea cycle detoxifying ammonia, and the citrulline-nitric oxide (NO) cycle producing NO. ASL-deficient patients present argininosuccinic aciduria characterised by hyperammonaemia, multiorgan disease and neurocognitive impairment despite treatment aiming to normalise ammonaemia without considering NO imbalance. Here we show that cerebral disease in argininosuccinic aciduria involves neuronal...
Paper Details
Title
Argininosuccinic aciduria fosters neuronal nitrosative stress reversed by Asl gene transfer
Published Date
Aug 29, 2018
Volume
9
Issue
1
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