Haemoglobin (Hb) J-Sardegna [α50(CE8)His → Asp] is a haemoglobin variant characteristic of subjects from the island of Sardinia. Here we report a study of the functional properties of both fetal and adult Hb J-Sardegna. The results indicate that adult Hb J-Sardegna displays an oxygen affinity that is higher than that of adult Hb only in the presence of 2,3-diphosphoglycerate (2,3-DPG). On the contrary, at 20 °C, the oxygen affinity of fetal Hb J-Sardegna is identical to that of normal fetal haemoglobin, both in the presence and in the absence of 2,3-DPG. A significant difference between these two systems (i.e. a higher oxygen affinity of fetal Hb J-Sardegna) shows up very clearly only when temperature is increased to 37 °C. Hence in fetal Hb, the main effect of the amino acid substitution is a decrease in the overall enthalpy change of oxygenation. The results outline the role of the α1-β1 interface in assessing the thermodynamics of oxygen binding. The functional properties of both adult and fetal Hb J-Sardegna have been interpreted at the structural level in light of the results obtained by a computational modelling approach performed in comparison with HbA and Hb Aichi, a variant characterized by a different mutation [α50(CE8)His → Arg] at the same position.
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Research Article|
February 08 2000
Adult and fetal haemoglobin J-Sardegna [α50(CE8)His→Asp]: functional and molecular modelling studies
Marcella CORDA;
Marcella CORDA
*Department of Biochemistry and Human Physiology, University of Cagliari, 09042 Monserrato Cagliari, Italy
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Maria C. DE ROSA;
Maria C. DE ROSA
†Institute of Chemistry and Clinical Chemistry, Catholic University of Rome, CNR - Receptor Chemistry Center of Rome, L. go F. Vito 1, 00168 Rome, Italy
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Maria G. PELLEGRINI;
Maria G. PELLEGRINI
*Department of Biochemistry and Human Physiology, University of Cagliari, 09042 Monserrato Cagliari, Italy
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Maria T. SANNA;
Maria T. SANNA
*Department of Biochemistry and Human Physiology, University of Cagliari, 09042 Monserrato Cagliari, Italy
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Alessandra OLIANAS;
Alessandra OLIANAS
*Department of Biochemistry and Human Physiology, University of Cagliari, 09042 Monserrato Cagliari, Italy
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Antonella FAIS;
Antonella FAIS
*Department of Biochemistry and Human Physiology, University of Cagliari, 09042 Monserrato Cagliari, Italy
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Laura MANCA;
Laura MANCA
‡Department of Physiological and Biochemical Sciences, University of Sassari, Corso Angioj 4, 07100 Sassari, Italy
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Bruno MASALA;
Bruno MASALA
‡Department of Physiological and Biochemical Sciences, University of Sassari, Corso Angioj 4, 07100 Sassari, Italy
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Bruno ZAPPACOSTA;
Bruno ZAPPACOSTA
†Institute of Chemistry and Clinical Chemistry, Catholic University of Rome, CNR - Receptor Chemistry Center of Rome, L. go F. Vito 1, 00168 Rome, Italy
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Silvana FICARRA;
Silvana FICARRA
§Department of Organic and Biological Chemistry, University of Messina, Salita Sperone 31, 98010 Messina, Italy
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Massimo CASTAGNOLA;
Massimo CASTAGNOLA
*Department of Biochemistry and Human Physiology, University of Cagliari, 09042 Monserrato Cagliari, Italy
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Bruno GIARDINA
Bruno GIARDINA
1
†Institute of Chemistry and Clinical Chemistry, Catholic University of Rome, CNR - Receptor Chemistry Center of Rome, L. go F. Vito 1, 00168 Rome, Italy
1To whom correspondence should be addressed (e-mail b.giardina@;uniserv.ccr.rm.cnr.it).
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Publisher: Portland Press Ltd
Received:
July 26 1999
Revision Received:
October 11 1999
Accepted:
November 18 1999
Online ISSN: 1470-8728
Print ISSN: 0264-6021
The Biochemical Society, London © 2000
2000
Biochem J (2000) 346 (1): 193–199.
Article history
Received:
July 26 1999
Revision Received:
October 11 1999
Accepted:
November 18 1999
Citation
Marcella CORDA, Maria C. DE ROSA, Maria G. PELLEGRINI, Maria T. SANNA, Alessandra OLIANAS, Antonella FAIS, Laura MANCA, Bruno MASALA, Bruno ZAPPACOSTA, Silvana FICARRA, Massimo CASTAGNOLA, Bruno GIARDINA; Adult and fetal haemoglobin J-Sardegna [α50(CE8)His→Asp]: functional and molecular modelling studies. Biochem J 15 February 2000; 346 (1): 193–199. doi: https://doi.org/10.1042/bj3460193
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