X-Ray and Solubility Studies of the Hæmoglobin of Sickle-Cell Anæmia Patients

Perutz, R R; Liquori, A. M.; Eirich, F. R.

doi:10.1038/167929a0

Cited by 146 publications

(49 citation statements)

References 6 publications

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“…The framework of parallel rods was torn apart as swelling increased, but the destruction appeared to be time consuming. HbS in the reduced state is relatively insoluble [14], and the organized bundles of rods appeared to resist the attack by increased intracellular water. Thus, damaged sickled cells were not immediately converted to ghosts, but revealed all stages of transformation.…”

Section: Discussionmentioning

confidence: 99%

Observations on the Mechanism of Erythrocyte Sickling

White

1969

Pediatr Res

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ExtractRecent studies have shown that the fundamental mechanism underlying the distortion of sickled erythrocytes is the conversion of reduced sickle cell hemoglobin (HbS) into rods. The abnormal molecules appear to stack into 65 A filaments that, through parallel association, form twisted rods 180-190 A in diameter. I n the present investigation, the effect of hemolysis on parallel bundles of rods in sickled cells has been examined. Hemolysis was found to destroy the framework of rods in the reverse stages offormation.The rods broke down into filaments, which disappeared into amorphous debris. T o further characterize the nature of the sickling phenomenon, the structure of hemoglobin crystals formed in canine erythrocytes by freeze-thaw was examined. The structure differed significantly from rods ofsickled hemoglobin. Results of these investigations support the concept that sickling is a process of sol-gel transformation, not of intracellular crystallization.

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Section: Discussionmentioning

confidence: 99%

Observations on the Mechanism of Erythrocyte Sickling

White

1969

Pediatr Res

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“…On the basis of unpublished data, Nalbandian and Kessler originally observed (5) that the Sickledex test was either identical with or derived from the Itano solubility test (6,7). This test wa: developed by Itano to distinguish between hemoglobins S and D. Guided by studies by Perutz (8,9), Itano observed that deoxygenated hemoglobin S had a uniquely low level of solubility in 2.28 M phosphate buffer system. Additional unpublished studies by Nalbandian and Henry disclosed that the working Sickledex solution contained phosphate in approximately 2.3 M concentrations, probably a quantity of sodium hydrosulfite (Na2S 2 0 4 ), and a hemolyzing agent considered to be saponin.…”

Section: Discussionmentioning

confidence: 99%

An Automated Screening Method for the Specific Detection of Homozygous and Heterozygous S Hemoglobin

Henry¹,

Nalbandlan²,

Nichols³

et al. 1970

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“…They are widely used as electrical insulation, protective clothing, straps, cables, and aerospace materials. On the other hand, wholly aromatic LC polymers have rarely been synthesized from bio-derived monomers, but have gained recognition in the field of sustainability science for many natural LC polymers such as DNA [5,6], RNA [7], hemoglobin S (HbS) [8], F-actin [9], and polysaccharides [10,11]. We have already reported the polymerization of trans-4-hydroxycinnamic acid (4HCA: p-coumaric acid) as a phytomonomer, which exists in plant cell walls as an intermediate metabolite of lignin and is produced by photosynthetic bacteria [12].…”

Section: Open Accessmentioning

confidence: 99%

Polarized Emission of Wholly Aromatic Bio-Based Copolyesters of a Liquid Crystalline Nature

Kan

Kaneko

2011

Polymers

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Abstract:A novel thermotropic liquid crystalline polymers poly{3-benzylidene amino-4-hydroxybenzoic acid (3,4-BAHBA)-co-trans-4-hydroxycinnamic acid (4HCA: trans-coumaric acid)} (Poly(3,4-BAHBA-co-4HCA)), was synthesized by the thermal polycondensation of 4HCA and 3,4-BAHBA, which was synthesized by a reaction of 3-amino-4-hydroxybenzoic acid (3,4-AHBA) with benzaldehyde. When the 4HCA compositions of Poly(3,4-BAHBA-co-4HCA)s were above 55 mol%, the copolymers showed a nematic, liquid crystalline phase. Differential scanning calorimetry (DSC) measurements of the copolymers showed a high glass transition temperature of more than 100 °C, sufficient for use in engineering plastics. Furthermore, the copolymers showed photoluminescence in an N-methylpyrrolidone (NMP) solution under ultraviolet (UV) light with a wavelength of 365 nm. Oriented film of Poly(3,4-BAHBA-co-4HCA) with a 4HCA composition of 75 mol% emitted polarized light, which was confirmed by fluorescent spectroscopy equipped with parallel and crossed polarizers.

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X-Ray and Solubility Studies of the Hæmoglobin of Sickle-Cell Anæmia Patients

Cited by 146 publications

References 6 publications

Observations on the Mechanism of Erythrocyte Sickling

Observations on the Mechanism of Erythrocyte Sickling

An Automated Screening Method for the Specific Detection of Homozygous and Heterozygous S Hemoglobin

Polarized Emission of Wholly Aromatic Bio-Based Copolyesters of a Liquid Crystalline Nature

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