Propionylation is a post-translational modification of proteins, in which a propionyl-group is added to a lysine amino acid of a protein. Propionylation participates in crucial biological processes, including metabolic processes and cellular stress response.[1]
Lysine propionylation was first identified on histone proteins,[2] and since has also been identified on other proteins.[3] Histone propionylation is a mark of active chromatin.[4] The substrate for protein propionylation is propionyl-CoA. Propionyl-CoA in the cell is metabolised by the enzyme propionyl-CoA carboxylase. Accumulation of propionyl-CoA leads to increased protein propionylation.[5]
In patients with propionic acidemia, a rare autosomal recessive metabolic disorder, propionyl-CoA levels elevated and increased propionylation,[6] which might contribute to the pathology in these patients.[5]