Title : The lysine catabolite saccharopine impairs development by disrupting mitochondrial homeostasis.

Pub. Date : 2019 Feb 4

PMID : 30573525






4 Functional Relationships(s)
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1 In Caenorhbditis elegans, mutations in the saccharopine dehydrogenase (SDH) domain of the bi-functional enzyme alpha-aminoadipic semialdehyde synthase AASS-1 greatly elevate the lysine catabolic intermediate saccharopine, which causes mitochondrial damage by disrupting mitochondrial dynamics, leading to reduced adult animal growth. saccharopine aminoadipate-semialdehyde synthase Mus musculus
2 Importantly, genetic inactivation of genes that raise the mitochondrial saccharopine precursors lysine and alpha-ketoglutarate strongly suppresses SDH mutation-induced saccharopine accumulation and mitochondrial abnormalities in C. elegans Thus, adequate saccharopine catabolism is essential for mitochondrial homeostasis. saccharopine aminoadipate-semialdehyde synthase Mus musculus
3 Importantly, genetic inactivation of genes that raise the mitochondrial saccharopine precursors lysine and alpha-ketoglutarate strongly suppresses SDH mutation-induced saccharopine accumulation and mitochondrial abnormalities in C. elegans Thus, adequate saccharopine catabolism is essential for mitochondrial homeostasis. saccharopine aminoadipate-semialdehyde synthase Mus musculus
4 Importantly, genetic inactivation of genes that raise the mitochondrial saccharopine precursors lysine and alpha-ketoglutarate strongly suppresses SDH mutation-induced saccharopine accumulation and mitochondrial abnormalities in C. elegans Thus, adequate saccharopine catabolism is essential for mitochondrial homeostasis. saccharopine aminoadipate-semialdehyde synthase Mus musculus