Impaired monocyte function in glycogen storage disease type Ib.
Phagocyte (neutrophil and monocyte) function was evaluated in a boy with glycogen storage disease type Ib. Neutrophils were found to be defective in motility and respiratory burst and monocytes showed a defect in respiratory burst but not in motility. These results suggested that the glucose-6-phosphate transport system plays a role in the function of neutrophils and monocytes and that these two phagocytes are different from each other in their energy metabolism for motility.
Ueno
N
N
Tomita
M
M
Ariga
T
T
Ohkawa
M
M
Nagano
S
S
Takahashi
Y
Y
Arashima
S
S
Matsumoto
S
S
D000293:Adolescent; D001853:Bone Marrow / Q000473:pathology; D002478:Cells, Cultured; D005953:Glycogen Storage Disease Type I / Q000175:diagnosis / Q000503:physiopathology*; D006801:Humans; D006942:Hypergammaglobulinemia / Q000276:immunology; D007070:Immunoglobulin A / Q000032:analysis; D007074:Immunoglobulin G / Q000032:analysis; D007075:Immunoglobulin M / Q000032:analysis; D008297:Male; D009000:Monocytes / Q000502:physiology*; D010587:Phagocytosis
D007070:Immunoglobulin A; D007074:Immunoglobulin G; D007075:Immunoglobulin M