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These pathological features are accompanied by synaptic loss, chronic neuroinflammation, mitochondrial dysfunction, oxidative stress, and progressive neuronal degeneration (Wang et al., 2020

[DOI] [PubMed] [Google Scholar] 91.Su CL, Sztalryd C, Contreras JA, Holm C, Kimmel AR, Londos C

BoSFaddaMFedeleDPellegriniMGhigoEPellegriniNet al

In addition, deletion of the GSH1 geneencoding glutamylcysteine synthetase (GCS), the enzyme catalysing the first step in glutathione synthesiswas shown to suppress an Ero1 conditional mutant, again indicating that GSH provides an additional load on Ero1 activity (Frand & Kaiser, 1998)

J Extracell Vesicles

Haemoglobin levels normalise over 68 weeks