The recent report of the oxidation of beta141 Leu in a New Zealand family with Hb Atlanta [beta75(E19)Leu-->Pro] prompted us to reinvestigate the original Hb Atlanta case from Georgia. Tryptic peptide maps showed that the modified beta(Co)T-14 peptide was present together with beta(A)T-14 in isopropanol precipitates of Hb Atlanta. Amino acid analysis confirmed that beta(Co)T-14 lacked leucine and mass spectrometry indicated that it had an increased mass of 16 Daltons. These findings support the proposition that the beta75 Leu-->Pro substitution in the E helix is the direct cause of the beta141 Leu oxidation.