Neutropenia and impaired neutrophil migration in type IB glycogen storage disease

Arthur L. Beaudet, Donald C. Anderson, Virginia V. Michels, William J. Arion, Alex J. Lange

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133 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)906-910
Number of pages5
JournalThe Journal of pediatrics
Issue number6
StatePublished - Dec 1980

Bibliographical note

Funding Information:
From the Departments of Pediatrics and Cell Biology, Baylor College of Medicine and Howard Hughes Medical Institute; and the Department of Physiology, Biochemistry and Pharmacology, New York State College of Veterinary Medicine, Cornell University. Supported by United States Public Health Service Grants RR-O0188, AM-19625, and GM-25397," and by the United States Department of Agriculture support for the Children's Nutrition and Research Center Texas Children's Hospital. *Reprint address: Department of Pediatrics, Baylor College of Medicine, Houston, Texas 77030.


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