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The NDUFS4 knockout mouse phenotype appears like the human Complex I shortage Leigh Syndrome. The permanent succination of healthy protein thiols by fumarate is raised in select areas of the NDUFS4 KO brain affected by neurodegeneration, suggesting a mechanistic duty in neurodegenerative decline. Succination of DLST lowered KGDHC task in the brainstem and olfactory light bulb of KO mice. HighlightsO_LICysteine succination by fumarate is increased in version of Complex I deficiency \ nC_LIO_LISuccination of DLST impairs KGDHC task, limits SLP, and reduces lysine succinylation \ nC_LIO_LIIrreversible succination drives neuropathology independent of the bioenergetic problem \ nC_LI.
Source link: https://doi.org/10.1101/2020.01.09.900514
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