Amelioration of Neutrophil Membrane Function Underlies Granulocyte-Colony Stimulating Factor Action in Glycogen Storage Disease 1b
| Autor: | Elisabetta Riva, Alfredo Gorio, A.M. Di Giulio, Elena Lesma, M. Giovannini |
|---|---|
| Rok vydání: | 2005 |
| Předmět: |
Adult
Blood Glucose medicine.medical_specialty Neutropenia Adolescent Neutrophils Immunology Glycogen Storage Disease Type I Granulocyte Biology Focal adhesion Leukocyte Count 03 medical and health sciences 0302 clinical medicine 030225 pediatrics Internal medicine Granulocyte Colony-Stimulating Factor medicine Humans Immunology and Allergy Glycogen storage disease Lactic Acid 030212 general & internal medicine Phosphorylation Child Pharmacology Cell adhesion molecule Cell Membrane Metabolic disorder Age Factors Infant Protein-Tyrosine Kinases medicine.disease Actins Recombinant Proteins Granulocyte colony-stimulating factor Treatment Outcome Endocrinology medicine.anatomical_structure Child Preschool Focal Adhesion Kinase 1 Focal Adhesion Protein-Tyrosine Kinases Gelsolin |
| Zdroj: | Scopus-Elsevier |
| ISSN: | 2058-7384 |
| Popis: | Glycogen storage disease (GSD) 1b is a metabolic disorder characterized by a deficiency of glucose 6-phosphate transporter and neutrophil alterations, which are reduced in number and functionally impaired. The present study aimed at investigating neutrophil dysfunction correlating submembrane and cytoskeletal changes at different ages with or without granulocyte-colony stimulating factor (G-CSF) treatment. GSD1b neutrophils showed reduced expression and diffused localization of focal adhesion kinase (FAK) and actin. No abnormalities were observed in GSD1a patient neutrophils. Gelsolin was also slightly reduced in neutrophils of GSD1b patients. When patients were treated for at least 3 months with G-CSF, the neutrophil number and the expression of FAK and actin were significantly increased. Granulocyte colony-stimulating factor treatment was similarly effective when performed in 1 year old patients. FAK auto- and IL-8-mediated phosphorylations were already affected as early as 1 year of age. G-CSF treatment also improved this alteration. Our data suggest that neutrophil dysfunction in GSD1b patients might be related to functional impairment and disorganization of proteins of the sub-membrane apparatus, and that G-CSF treatment counteracts neutropenia and prevents the progressive alterations of neutrophil sub-membrane proteins. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|