Neutropenia and impaired neutrophil migration in type IB glycogen storage disease.
The association of neutropenia with type IB glycogen storage disease was observed in siblings. Biochemical studies of liver demonstrated a defect in glucose-6-phosphate transport. Neutrophil mobilization in vivo was impaired but the bone marrow was normal histologically. In vitro studies of neutrophils indicated a defect in random and directed cell migration. The studies suggest that a role for glucose-6-phosphate transport in neutrophils should be considered.
D000380:Agranulocytosis / Q000209:etiology*; D002634:Chemotaxis, Leukocyte; D005952:Glucose-6-Phosphatase / Q000032:analysis; D005953:Glycogen Storage Disease Type I / Q000097:blood* / Q000150:complications; D006801:Humans; D007223:Infant; D008099:Liver / Q000032:analysis; D008297:Male; D009503:Neutropenia / Q000209:etiology*; D009504:Neutrophils
D005952:Glucose-6-Phosphatase