Characterization of a new electrophoretically silent hemoglobin variant - Hb Saale OR alpha(2)beta(2) 84(EF8)Thr -> ala

Authors
Bisse, E Zorn, N Heinrichs, I Eigel, A Van Dorsselaer, A Wieland, H Kister, J Marden, MC
Citation
E. Bisse et al., Characterization of a new electrophoretically silent hemoglobin variant - Hb Saale OR alpha(2)beta(2) 84(EF8)Thr -> ala, J BIOL CHEM, 275(28), 2000, pp. 21380-21384
Citations number
21
Categorie Soggetti
Biochemistry & Biophysics
Journal title
JOURNAL OF BIOLOGICAL CHEMISTRY
ISSN journal
00219258 → ACNP
Volume
275
Issue
28
Year of publication
2000
Pages
21380 - 21384
Database
ISI
SICI code
0021-9258(20000714)275:28<21380:COANES>2.0.ZU;2-5
Abstract
A new abnormal hemoglobin was detected in a young German anemic patient by cation-exchange high performance liquid chromatography (HPLC), Using a comb ination of electrospray mass spectrometry, HPLC, direct sequencing, and fam ily screening with polymerase chain reaction/restriction digestion approach , we have characterized this hemoglobin variant as resulting from a Thr --> Ala replacement at beta 84(EF8). It could be separated neither by electrop horesis nor by isoelectric focusing. Hb Saale is slightly unstable, exhibit ing a moderate tendency to auto-oxidize. Functional properties and the hete rotropic interactions are similar to those of Hb A.