Glycogen storage disease type Ib neutrophils exhibit impaired cell adhesion and migration
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文摘
GSD-Ib deficient in G6PT is characterized by neutropenia in both blood and bone marrow. Neutrophil recruitment into peritoneal cavity is impaired in G6pt−/− and G-CSF-treated G6pt−/− mice. Neutrophils from G6pt−/− mice exhibit impaired cell migration and adhesion. The aberrant expression of β2 integrins, CD11b and CD11a may lead to impaired cell adhesion in G6pt −/− neutrophils.

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