Decreased osmotic fragility in heritable canine myopathy

✍ Scribed by Raymond H. Abhold; Gerald A. Hegreberg; James A. Magnuson

• Publisher: John Wiley and Sons
• Year: 1983
• Tongue: English
• Weight: 458 KB
• Volume: 15
• Category: Article
• ISSN: 0361-8609
• DOI: 10.1002/ajh.2830150103

✦ Synopsis

Osmotic fragility was examined in red blood cells from dogs with a heritable muscle disorder that clinically resembles a muscular dystrophy. Several erythrocyte abnormalities have been reported in patients with certain forms of muscular dystrophy and it is thought that these changes reflect genetically induced alterations in the plasma membrane. It is believed that the examination of erythrocytes may eventually lead to the understanding of membrane involvement in muscle disorders. In this study. the mean osmotic fragility was found to be significantly lower in affected cells than in normal cells. These diffcrcnces were maintained regardless of changes in incubation temperature (5". 20". o r 35°C) and pH (6.5. 7.0. 7.5, or 8.0). Quantitativc analysis of glycolytic metabolites and adenine nucleotide concentrations rcvcalcd little variance between erythrocytes from normal and affected animals. Similarly. thc pattern of membrane protein phosphorylation in intact erythrocytes from affcctcd animals did not differ from that observed when erythrocytes from normal animals were examined. Of the red cell indices measured. the erythrocyte count in affcctcd animals was moderately incrcased, but both the mean corpuscular volume and mean corpuscular hernoglobin content were significantly reduced. From these data it is concluded that the decrease in osmotic fragility cannot be explained by differences in cell metabolism or energy production. However. the decrease in affected cell mean corpuscular volume and mean corpuscular hemoglobin content may be correlated with the decrease in osmotic fragility in a manner similar to that observed in the hemolytic disorder of beta-thalassemia.