Temperature Effects on Cation Transport in Hereditary Stomatocytosis and Allied Disorders

Overview

Journal Int J Exp Pathol

Publisher Wiley

Specialty Pathology

Date 1999 Dec 22

PMID 10607015

Citations 4

Authors

S E Coles

G W Stewart

Affiliations

Soon will be listed here.

Abstract

The conditions known as 'hereditary stomatocytosis and allied syndromes' comprise a group of dominantly inherited human haemolytic anaemias characterized by a plasma membrane 'leak' to the univalent cations Na and K, an example of a small but growing group of diseases where pathology can be directly attributed to abnormal membrane transport. A number of case reports in the different variants have alluded to temperature-related phenomena, including loss of K on storage at room temperature (giving 'pseudohyperkalaemia') and lysis of cells when stored in the cold ('cryohydrocytosis'). This review collects together published studies of these temperature effects, which show very major differences in the 'leak' K transport. Two main variations on normal emerge: a 'shallow slope' type, in which the flux shows an abnormally low dependence on temperature in the range 37-20 degrees C, and 'high minimum', in which the minimum in this flux, which occurs in normal cells at 8 degrees C, is shifted up to 23 degrees C. These temperature studies provide a powerful method for phenotypic characterization.

Citing Articles

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The Molecular Basis for Altered Cation Permeability in Hereditary Stomatocytic Human Red Blood Cells.

Flatt J, Bruce L Front Physiol. 2018; 9:367.

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Pulmonary hypertension associated with chronic hemolytic anemia and other blood disorders.

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The GPA-dependent, spherostomatocytosis mutant AE1 E758K induces GPA-independent, endogenous cation transport in amphibian oocytes.

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PMID: 19907019 PMC: 2822494. DOI: 10.1152/ajpcell.00444.2009.

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