 n - myristoylation is a common form of co - translational protein fatty acylation resulting from the attachment of myristate to a required n - terminal glycine residue.1,2 we show that aberrantly acquired n - myristoylation of shoc2 , a leucine - rich repeat - containing protein that positively modulates ras - mapk signal flow,36 underlies a clinically distinctive condition of the neuro - cardio - facial - cutaneous disorders family . 
 twenty - five subjects with a relatively consistent phenotype previously termed noonan - like syndrome with loose anagen hair [ omim 607721]7 shared the 4a > g missense change ( ser2gly ) in shoc2 that introduces an n - myristoylation site , resulting in aberrant targeting of shoc2 to the plasma membrane and impaired translocation to the nucleus upon growth factor stimulation . 
 expression of shoc2s2 g 
 in vitro enhanced mapk activation in a cell type - specific fashion . 
 induction of shoc2s2 g in caenorhabditis elegans engendered protruding vulva , a neomorphic phenotype previously associated with aberrant signaling . 
 these results document the first example of an acquired n - terminal lipid modification of a protein causing human disease . 
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