Background:No known function for the amino acid cystathionine other than as an intermediate in cysteine synthesis. Results: Cystathionine prevents ER stress induced steatotic liver injury, acute tubular necrosis and apoptosis without changing induction of the unfolded protein response. Conclusion: Abolition of cystathionine synthesis may contribute to pathogenesis in homocystinuria. Significance: Cystathionine has therapeutic potential for disease states where ER stress is implicated.

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