Article
Hb Montefiore (126(H9)Asp-->Tyr). High oxygen affinity and loss of cooperativity secondary to C-terminal disruption.
The Journal of biological chemistry - 20 Sept 1996
Wajcman H, Kister J, Galactéros F, Spielvogel A, Lin M J, Vidugiris G J, Hirsch R E, Friedman J M, Nagel R L
Abstract excerpt
Hb Montefiore was found, in the heterozygous state, in a Puerto Rican female who had a slightly elevated total Hb level. Structural analysis revealed that Asp-alpha126 was replaced by Tyr. Hb Montefiore migrates close to HbF (at pH 8.6) and accounts for 20.3% of the hemolysate. Oxygen binding of red blood cells revealed a 40% decrease in the P50 (pH 7.4) and a low n value of 1.6 (normal: 2.6). Depletion of red...
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