glutathione synthetase gene Multiple congenital anomalies in two fetuses with glutathione‐synthetase deficit (GSS) - Jury - 2024 - Clinical Genetics RCSB PDB - 2HGS: HUMAN

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The equilibration that results leads to a collapse of the proton-motive force across the membrane and a colloid osmotic pressure that causes massive swelling of the mitochondria (McStay et al., 2002)

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In addition, in RP conditions the redox system is disturbed and as such for all postnatal days the [GSH]/[GSSG] ratio is sensitive to elements that govern the enzymatic reaction in which GSH reduces ROS