The First Japanese Case of Hb Santa Ana, an Unstable Abnormal Hemoglobin, Identified Rapidly by Electrospray Ionization Mass Spectrometry.
Open Access
- 1 January 1997
- journal article
- case report
- Published by Japanese Society of Internal Medicine in Internal Medicine
- Vol. 36 (5) , 365-370
- https://doi.org/10.2169/internalmedicine.36.365
Abstract
Two members of a family had chronic hemolytic anemia due to unstable hemoglobin. The abnormal β-chain with a molecular weight of 16 u smaller than the normal β-chain was found within 5 minutes by analysis using electrospray ionization mass spectrometry. The substitution of amino acid was also determined rapidly by this new strategy. The leucine at the 88th position of the normal β-chain was substituted by proline in the hemoglobin as in Hb Santa Ana. This is the first report of a Japanese case of Hb Santa Ana; the clinical course was similar to that in the previously reported cases.(Internal Medicine 36: 365-370, 1997)Keywords
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