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PMID:7518430

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Citation

Sanna, MT, Giardina, B, Scatena, R, Pellegrini, M, Olianas, A, Manca, L, Masala, B, Castagnola, M and Corda, M (1994) Functional alterations in adult and fetal hemoglobin Sassari Asp-alpha 126(H9)-->His. The role of alpha 1 alpha 2 contact. J. Biol. Chem. 269:18338-42

Abstract

The effects of pH, organic phosphates (2,3-diphosphoglycerate), and temperature on the functional properties of both adult and fetal hemoglobin Sassari alpha (Asp-126-->His) have been studied. The functional properties of the adult variant are characterized by the following: (i) an oxygen affinity higher than that of normal HbA in all the experimental conditions used; (ii) a dramatic reduction of homotropic interactions (n50 very close to unity); and (iii) a significant decrease of the effect of 2,3-diphosphoglycerate, which is 35% lower than that observed on HbA. The fetal variant shows an increased oxygen affinity compared with normal HbF and an almost abolished heme-heme interaction. The molecular basis of these functional differences is discussed in terms of the possible role played by the substitution of alpha (Asp-26-->His) on the stability of the R state of the molecule due to a decreased interaction at the level of alpha 1 alpha 2 contact.

Links

PubMed

Keywords

Adult; Amino Acid Sequence; Aspartic Acid; Female; Fetal Blood; Fetal Hemoglobin/chemistry; Fetal Hemoglobin/genetics; Fetal Hemoglobin/isolation & purification; Hemoglobin A/chemistry; Hemoglobin A/genetics; Hemoglobin A/isolation & purification; Hemoglobins, Abnormal/chemistry; Hemoglobins, Abnormal/genetics; Hemoglobins, Abnormal/isolation & purification; Heterozygote Detection; Histidine; Humans; Hydrogen-Ion Concentration; Infant, Newborn; Macromolecular Substances; Male; Molecular Sequence Data; Oxyhemoglobins/metabolism; Point Mutation

Significance

Annotations

Gene product Qualifier GO Term Evidence Code with/from Aspect Extension Notes Status

HUMAN:HBB

involved_in

GO:0015671: oxygen transport

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

HUMAN:HBB

involved_in

GO:0015670: carbon dioxide transport

ECO:0005547: biological system reconstruction evidence based on inference from background scientific knowledge used in manual assertion

P

Seeded From UniProt

complete

HUMAN:HBA

involved_in

GO:0015671: oxygen transport

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

HUMAN:HBA

involved_in

GO:0015670: carbon dioxide transport

ECO:0005547: biological system reconstruction evidence based on inference from background scientific knowledge used in manual assertion

P

Seeded From UniProt

complete

HUMAN:HBA

involved_in

GO:0015671: oxygen transport

ECO:0000304: author statement supported by traceable reference used in manual assertion

P

Seeded From UniProt

complete


See also

References

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