Posttranslational modification of beta 141 Leu associated with the beta 75(E19)Leu-->Pro mutation in Hb Atlanta.
The recent report of the oxidation of beta 141 Leu in a New Zealand family with Hb Atlanta [ beta 75(E19)Leu-->Pro] prompted us to reinvestigate the original Hb Atlanta case from Georgia. Tryptic peptide maps showed that the modified beta CoT-14 peptide was present together with beta AT-14 in isopropanol precipitates of Hb Atlanta. Amino acid analysis confirmed that beta CoT-14 lacked leucine and mass spectrometry indicated that it had an increased mass of 16 Daltons. These findings support the proposition that the beta 75 Leu-->Pro substitution in the E helix is the direct cause of the beta 141 Leu oxidation.
Brennan
S O
SO
Pathology Department, Christchurch Hospital, New Zealand.
Shaw
J G
JG
George
P M
PM
Huisman
T H
TH
D000595:Amino Acid Sequence; D005914:Globins / Q000235:genetics / Q000378:metabolism*; D006455:Hemoglobins, Abnormal / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D007930:Leucine / Q000031:analogs & derivatives* / Q000032:analysis; D013058:Mass Spectrometry; D008969:Molecular Sequence Data; D010084:Oxidation-Reduction; D010449:Peptide Mapping; D011499:Protein Processing, Post-Translational
D006455:Hemoglobins, Abnormal; C035046:hemoglobin Atlanta; C024584:beta-hydroxyleucine; D005914:Globins; D007930:Leucine