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Literature DB >> 6578930

Impaired metabolic function of polymorphonuclear leukocytes in glycogen storage disease Ib.

Abstract

To elucidate the basis for the recurrent infections in patients with glycogen storage disease (GSD) Ib we tested polymorphonuclear leukocyte (PMN) function in one patient. Bactericidal capacity and phagocytosis-induced O2 consumption were reduced. Also, phorbol myristate acetate-stimulated superoxide production and glucose oxidation through the hexose monophosphate shunt were diminished compared to control subjects. Therefore it could be speculated that in PMN of patients with GSD Ib, glucose-6-phosphate has no access to the enzymes of the hexose monophosphate shunt due to a transport-related defect as shown for glucogenesis in hepatocytes.

Entities: Chemical Disease Species

Mesh：

Year: 1983 PMID： 6578930 DOI： 10.1007/bf00442674

Source DB: PubMed Journal: Eur J Pediatr ISSN： 0340-6199 Impact factor: 3.183

8 in total

1. A new variant of glycogen storage disease type I probably due to a defect in the glucose-6-phosphate transport system.

Authors: K Narisawa; Y Igarashi; H Otomo; K Tada
Journal: Biochem Biophys Res Commun Date: 1978-08-29 Impact factor: 3.575

2. Superoxide generation by digitonin-stimulated guinea pig granulocytes. A basis for a continuous assay for monitoring superoxide production and for the study of the activation of the generating system.

Authors: H J Cohen; M E Chovaniec
Journal: J Clin Invest Date: 1978-04 Impact factor: 14.808

3. Nitroblue-tetrazolium test for the detection of chronic granulomatous disease--technical modification.

Authors: E Preisig; W H Hitzig
Journal: Eur J Clin Invest Date: 1971-09 Impact factor: 4.686

4. Type Ib glycogen storage disease is caused by a defect in the glucose-6-phosphate translocase of the microsomal glucose-6-phosphatase system.

Authors: A J Lange; W J Arion; A L Beaudet
Journal: J Biol Chem Date: 1980-09-25 Impact factor: 5.157

5. Is type 1b glycogenosis related to an anomeric preference for glucose-6-phosphate uptake by hepatic microsomes?

Authors: P P Kamoun
Journal: Med Hypotheses Date: 1980-11 Impact factor: 1.538

6. Differentiation between glycogenosis types Ia and Ib by measurement of extra respiration during phagocytosis by polymorphonuclear leukocytes?

Authors: K Heyne; M Gahr
Journal: Eur J Pediatr Date: 1980 Impact factor: 3.183

7. Neutropenia and impaired neutrophil migration in type IB glycogen storage disease.

Authors: A L Beaudet; D C Anderson; V V Michels; W J Arion; A J Lange
Journal: J Pediatr Date: 1980-12 Impact factor: 4.406

8. Recurrent infection in glycogenosis type Ib: abnormal neutrophil motility related to impaired redistribution of adhesion sites.

Authors: D C Anderson; M L Mace; B R Brinkley; R R Martin; C W Smith
Journal: J Infect Dis Date: 1981-03 Impact factor: 5.226

8 in total

13 in total

Impaired metabolic function of polymorphonuclear leukocytes in glycogen storage disease Ib.

1. A new variant of glycogen storage disease type I probably due to a defect in the glucose-6-phosphate transport system.

2. Superoxide generation by digitonin-stimulated guinea pig granulocytes. A basis for a continuous assay for monitoring superoxide production and for the study of the activation of the generating system.

3. Nitroblue-tetrazolium test for the detection of chronic granulomatous disease--technical modification.

4. Type Ib glycogen storage disease is caused by a defect in the glucose-6-phosphate translocase of the microsomal glucose-6-phosphatase system.

5. Is type 1b glycogenosis related to an anomeric preference for glucose-6-phosphate uptake by hepatic microsomes?

6. Differentiation between glycogenosis types Ia and Ib by measurement of extra respiration during phagocytosis by polymorphonuclear leukocytes?

7. Neutropenia and impaired neutrophil migration in type IB glycogen storage disease.

8. Recurrent infection in glycogenosis type Ib: abnormal neutrophil motility related to impaired redistribution of adhesion sites.

1. Impaired glucose transport in polymorphonuclear leukocytes in glycogen storage disease Ib.

2. Normal polymorphonuclear neutrophil function in a case of glycogen storage disease type Ib.

3. Neutrophil metabolic dysfunction in genetically heterogeneous patients with glycogen storage disease type 1b.

4. Secondary amyloidosis in glycogen storage disease type Ib.

5. Intact natural killer activity in glycogenosis type Ib.

6. Impaired monocyte function in glycogen storage disease type Ib.

7. Impaired carbohydrate metabolism of polymorphonuclear leukocytes in glycogen storage disease Ib.

8. Postnatal regression of glucose transport in a patient with glycogen storage disease type 1b.

9. Chronic inflammatory bowel disease in glycogen storage disease type 1B.

10. Glycogen storage disease type Ib: familial bleeding tendency.