Article

A PARP inhibitor, rucaparib, improves cardiac dysfunction in <i>ADP-ribose-acceptor hydrolase 3</i> ( <i>Arh3</i> ) deficiency

2023-02-07

Abstract excerpt

<h4>Aims</h4> Patients with ADP-ribose-acceptor hydrolase 3 ( ARH3 ) deficiency exhibit stress-induced childhood-onset neurodegeneration with ataxia and seizures (CONDSIAS). ARH3 degrades protein-linked poly(ADP- ribose) (PAR) synthesized by poly(ADP-ribose)polymerase (PARP)-1 during oxidative stress, leading to cleavage of the ADP-ribose linked to protein. ARH3 deficiency leads to excess accumulation of PAR,...

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Literature Corpus work: f220be5c-b4c8-58de-ae74-a71e890ed03f
DOI: 10.1101/2023.02.07.527369

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