Anion Transport and 2,3-Diphosphoglycerate in Cystic Fibrosis Red Blood Cells

Berghout, Alexander; Bender, S. W.

doi:10.1203/00006450-198418100-00021

Cited by 1 publication

(2 citation statements)

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“…Cl − crosses the cell membrane mainly through band 3 channels. The reduction of the number of CFTR molecules in patients (e. g. [53]) does not affect this exchange [24]. Cl − concurs with BPG for the same binding sites on Hb [54] but its affinity is lower and the small decrease of its concentration in CF is compensated for by increased [BPG].…”

Section: Discussionmentioning

confidence: 99%

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Hemoglobin Oxygen Affinity in Patients with Cystic Fibrosis

et al. 2014

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In patients with cystic fibrosis lung damages cause arterial hypoxia. As a typical compensatory reaction one might expect changes in oxygen affinity of hemoglobin. Therefore position (standard half saturation pressure P50st) and slope (Hill’s n) of the O2 dissociation curve as well as the Bohr coefficients (BC) for CO2 and lactic acid were determined in blood of 14 adult patients (8 males, 6 females) and 14 healthy controls (6 males, 8 females). While Hill’s n amounted to approximately 2.6 in all subjects, P50st was slightly increased by 1mmHg in both patient groups (controls male 26.7±0.2, controls female 27.0±0.1, patients male 27.7±0.5, patients female 28.0±0.3 mmHg; mean and standard error, overall p<0.01). Main cause was a rise of 1–2 µmol/g hemoglobin in erythrocytic 2,3-biphosphoglycerate concentration. One patient only, clearly identified as an outlier and with the mutation G551D, showed a reduction of both P50st (24.5 mmHg) and [2,3-biphosphoglycerate] (9.8 µmol/g hemoglobin). There were no differences in BCCO2, but small sex differences in the BC for lactic acid in the controls which were not detectable in the patients. Causes for the right shift of the O2 dissociation curve might be hypoxic stimulation of erythrocytic glycolysis and an increased red cell turnover both causing increased [2,3-biphosphoglycerate]. However, for situations with additional hypercapnia as observed in exercising patients a left shift seems to be a more favourable adaptation in cystic fibrosis. Additionally when in vivo PO2 values were corrected to the standard conditions they mostly lay left of the in vitro O2 dissociation curve in both patients and controls. This hints to unknown fugitive factors influencing oxygen affinity.

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Section: Discussionmentioning

confidence: 99%

“…Compensatory reactions of oxygen affinity in cystic fibrosis have been investigated rarely. Slight right shifts of the standard ODC, characterized by a rise of P 50 st and caused by increased [BPG], were detected by some authors [18], [23], while others found unchanged [BPG] or P 50 st [19], [24].…”

Section: Introductionmentioning

confidence: 99%