WDYHV1 mediates the side-chain deamidation of N-terminal glutamine residues to glutamate, an important step in N-end rule pathway of protein degradation. Conversion of the resulting N-terminal glutamine to glutamate renders the protein susceptible to arginylation, polyubiquitination and degradation as specified by the N-end rule. Does not act on substrates with internal or C-terminal glutamine and does not act on non-glutamine residues in any position. Does not deaminate acetylated N-terminal glutamine. With the exception of proline, all tested second-position residues on substrate peptides do not greatly influence the activity. In contrast, a proline at position 2, virtually abolishes deamidation of N-terminal glutamine.
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C8orf32; N-terminal Gln amidase; nt(Q)-amidase; Protein N-terminal glutamine amidohydrolase; Protein NH2-terminal glutamine deamidase; WDYHV motif containing 1; WDYHV motif-containing protein 1