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

doi:10.1016/S0022-3476(80)80418-5

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

Medicine - Endocrine and Diabetes Division

Research output: Contribution to journal › Article › peer-review

125 Scopus citations

Abstract

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 language	English (US)
Pages (from-to)	906-910
Number of pages	5
Journal	The Journal of pediatrics
Volume	97
Issue number	6
DOIs	https://doi.org/10.1016/S0022-3476(80)80418-5
State	Published - 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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title = "Neutropenia and impaired neutrophil migration in type IB glycogen storage disease",

abstract = "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.",

author = "Beaudet, {Arthur L.} and Anderson, {Donald C.} and Michels, {Virginia V.} and Arion, {William J.} and Lange, {Alex J.}",

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.",

year = "1980",

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T1 - Neutropenia and impaired neutrophil migration in type IB glycogen storage disease

AU - Beaudet, Arthur L.

AU - Anderson, Donald C.

AU - Michels, Virginia V.

AU - Arion, William J.

AU - Lange, Alex J.

N1 - 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.

PY - 1980/12

Y1 - 1980/12

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