In Ataxia-Telangiectasia, Oral Betamethasone Administration Ameliorates Lymphocytes Functionality through Modulation of the IL-7/IL-7Rα Axis Paralleling the Neurological Behavior: A Comparative Report of Two Cases.
Administration, Oral
Anti-Inflammatory Agents
/ therapeutic use
Ataxia Telangiectasia
/ drug therapy
Attention Deficit and Disruptive Behavior Disorders
/ drug therapy
Betamethasone
/ therapeutic use
Child, Preschool
Female
Humans
Interleukin-7
/ metabolism
Lymphocyte Activation
/ drug effects
Lymphocytes
/ immunology
Male
Microarray Analysis
Receptors, Interleukin-7
/ metabolism
Signal Transduction
/ drug effects
Ataxia-Teleangiectasia
Il-7/IL-7Rα axis
Il-7Rα
betamethasone
intracellular trafficking
receptor recycling
Journal
Immunological investigations
ISSN: 1532-4311
Titre abrégé: Immunol Invest
Pays: England
ID NLM: 8504629
Informations de publication
Date de publication:
Feb 2021
Feb 2021
Historique:
pubmed:
14
5
2020
medline:
1
10
2021
entrez:
14
5
2020
Statut:
ppublish
Résumé
Ataxia-Telangiectasia (A-T) is characterized by cerebellar neurodegeneration and immunodeficiency. Recent studies suggest that very low glucocorticoids (GCs) doses may help improve A-T neurological phenotype in some patients. Interestingly, in GCs studies an unexpected improvement of lymphocytes proliferation in some A-T patients has been observed. GCs are able to upregulate IL-7 Rα expression and rescue it from the recycling. In this study, we compared several immunological functions, including PBMC proliferative responses, cell activation events and IL-7/IL-7 Rα axis functionality, with the neurological behavior during an
Identifiants
pubmed: 32397775
doi: 10.1080/08820139.2020.1761379
doi:
Substances chimiques
Anti-Inflammatory Agents
0
Interleukin-7
0
Receptors, Interleukin-7
0
interleukin-7 receptor, alpha chain
0
Betamethasone
9842X06Q6M
Types de publication
Case Reports
Comparative Study
Journal Article
Langues
eng
Sous-ensembles de citation
IM