NAA10

NAA10 (N-alpha-acetyltransferase 10, ARD1A) is the catalytic subunit of the NatA N-terminal acetyltransferase complex, the major co-translational N-terminal acetyltransferase in human cells. In complex with its auxiliary subunit NAA15, which anchors the enzyme to the ribosome, NAA10 transfers an acetyl group from acetyl-CoA to the free alpha-amino group of nascent polypeptide N-termini that begin with small residues (Ser, Ala, Thr, Gly, Cys, Val) exposed after initiator-methionine excision. This irreversible modification affects protein folding, stability, complex assembly, targeting and degradation. NatA activity is further modulated by the associated factors HYPK and NAA50 (the NatE catalytic subunit). NAA10 acts predominantly in the cytoplasm on ribosome-associated nascent chains, with an additional nuclear pool. The free (NAA15-unbound) form has been reported to perform internal (lysine) acetylation of selected substrates (e.g. HIF1A, HSPA1A/B, histone H4), and NAA10 has been implicated in several context-dependent regulatory roles. NAA10 is X-linked (Xq28); pathogenic variants cause Ogden syndrome and related N-terminal acetyltransferase deficiency disorders.

Disorders

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