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Titolo:
HBC COMPOUND HETEROZYGOTES [HBC HB RIYADH AND HBC/HB N-BALTIMORE] WITH OPPOSING EFFECTS UPON HBC CRYSTALLIZATION/
Autore:
HIRSCH RE; WITKOWSKA HE; SHAFER F; LIN MJ; BALAZS TC; BOOKCHIN RM; NAGEL RL;
Indirizzi:
YESHIVA UNIV ALBERT EINSTEIN COLL MED,DEPT MED,DIV HEMATOL,1300 MORRIS PK AVE,ULLMANN 923 BRONX NY 10461 YESHIVA UNIV ALBERT EINSTEIN COLL MED,DEPT PHYSIOL & BIOPHYS BRONX NY10461 YESHIVA UNIV ALBERT EINSTEIN COLL MED,DEPT ANAT & STRUCT BIOL BRONX NY 10461 CHILDRENS HOSP OAKLAND,RES INST,DEPT HEMATOL ONCOL OAKLAND CA 94609
Titolo Testata:
British Journal of Haematology
fascicolo: 2, volume: 97, anno: 1997,
pagine: 259 - 265
SICI:
0007-1048(1997)97:2<259:HCH[HR>2.0.ZU;2-T
Fonte:
ISI
Lingua:
ENG
Soggetto:
HEMOGLOBIN-C CRYSTALLIZATION; INTERMOLECULAR CONTACTS; MASS-SPECTROMETRY; CRYSTAL-FORMATION; FIBERS; EKTACYTOMETRY; ERYTHROCYTES; ACCELERATION; DISEASE;
Keywords:
HEMOGLOBIN CRYSTAL; RED CELLS; HBS; MASS SPECTROMETRY; ABNORMAL HEMOGLOBINS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
28
Recensione:
Indirizzi per estratti:
Citazione:
R.E. Hirsch et al., "HBC COMPOUND HETEROZYGOTES [HBC HB RIYADH AND HBC/HB N-BALTIMORE] WITH OPPOSING EFFECTS UPON HBC CRYSTALLIZATION/", British Journal of Haematology, 97(2), 1997, pp. 259-265

Abstract

Compound heterozygotes of variant haemoglobins (Hbs) with HbC, with or without novel phenotypic changes, have provided insight into the molecular basis of the interacting haemoglobins and information concerning the role of specific residues in the crystallization of oxy HbC. A high phosphate buffer system has proved useful for studying the effectsof variant haemoglobins (naturally coexisting with HbC in the red cell) on the oxy HbC crystallization process and has led us to conclude that beta 87 and beta 73 are contact sites of the oxy HbC crystal. We now present investigations from two HbC compound heterozygotes which exhibit opposing effects upon HbC crystallization: HbC/Hb N-Baltimore (beta 95 Lys --> Glu) and HbC/Hb Riyadh (beta 120 Lys-->Asn). The latterinhibits the in vitro crystallization of HbC, explaining the lack of erythrocyte abnormalities (with the exception of microcytosis) in the doubly heterozygous infant. In contrast, Hb N-Baltimore accelerates the crystallization of HbC, contributing to multiple abnormalities in red cell morphology, albeit in the absence of morbidity. We conclude that (1) beta 120 and beta 95 are additional contact sites in the crystal, and (2) the HbC/Hb Riyadh haemoglobinopathy demonstrates that crystallization may not be required for the generation of the observed microcytosis and increased red cell density in HbC-containing red cells.

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Documento generato il 21/09/20 alle ore 06:32:32