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Spectrin modifications in a heterozygous case of both hereditary elliptocytosis and β-thalassemia

✍ Scribed by Dr. Sara Streichman; Ethy Herz; Ilana Tatarsky

Publisher
John Wiley and Sons
Year
1990
Tongue
English
Weight
504 KB
Volume
33
Category
Article
ISSN
0361-8609

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✦ Synopsis

The clinical and hematological parameters of a patient described here, who inherited the genes of both hereditary elliptocytosis (HE) and p-thalassemia, seem to reflect a mutual enhancement of the two diseases. The coexistence of the two pathologies is probably also responsible for the observed changes in spectrin: the appearance of an extra spectrin band between tetramers and dimers on denaturing gel electrophoresis and the metabolic-dependent reduction in spectrin amount. It is assumed that the instability of the skeletal network that results from the HE pathology caused increased exposure of the spectrin molecule to oxidative damage that usually occurs in thalassernic red cells. The products of such oxidation may have led to abnormal spectrin associations which finally resulted in the above changes.

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## Abstract Patients with β‐thalassemia trait have been reported to present lower plasma concentrations of low‐density lipoprotein (LDL) and lower frequencies of acute myocardial infarction than normal subjects. In this study, the metabolism of LDL was tested in 12 patients with heterozygous β‐thal