Back to Search
Start Over
Biological and functional characterization of bone marrow-derived mesenchymal stromal cells from patients affected by primary immunodeficiency
- Source :
- Scientific Reports, Scientific Reports, Vol 7, Iss 1, Pp 1-13 (2017)
- Publication Year :
- 2017
-
Abstract
- Mesenchymal stromal cells (MSCs) represent a key component of bone marrow (BM) microenvironment and display immune-regulatory properties. We performed a detailed analysis of biological/functional properties of BM-MSCs derived from 33 pediatric patients affected by primary immune-deficiencies (PID-MSCs): 7 Chronic Granulomatous Disease (CGD), 15 Wiskott-Aldrich Syndrome (WAS), 11 Severe Combined Immunodeficiency (SCID). Results were compared with MSCs from 15 age-matched pediatric healthy-donors (HD-MSCs). Clonogenic and proliferative capacity, differentiation ability, immunophenotype, immunomodulatory properties were analyzed. WB and RT-qPCR for CYBB, WAS and ADA genes were performed. All PID-MSCs displayed clonogenic and proliferative capacity, morphology and immunophenotype comparable with HD-MSCs. PID-MSCs maintained the inhibitory effect on T- and B-lymphocyte proliferation, except for decreased inhibitory ability of SCID-MSCs at MSC:PBMC ratio 1:10. While HD- and CGD-MSCs were able to inhibit monocyte maturation into immature dendritic cells, in SCID- and WAS-MSCs this ability was reduced. After Toll-like Receptor priming, PID-MSCs displayed in vitro an altered gene expression profile of pro- and anti-inflammatory soluble factors. PID-MSCs displayed lower PPARĪ³ levels and WAS- and SCID-MSCs higher levels of key osteogenic markers, as compared with HD-MSCs. Our results indicate that PID-MSCs may be defective in some functional abilities; whether these defects contribute to disease pathophysiology deserves further investigation.
- Subjects :
- 0301 basic medicine
Male
Adolescent
Cellular differentiation
Science
T-Lymphocytes
chronic granulomatous-disease
Wiskott-Aldrich syndrome
versus-host disease
Bone Marrow Cells
Biology
Lymphocyte Activation
Monocytes
Article
MSC
03 medical and health sciences
Immunophenotyping
Chronic granulomatous disease
medicine
Adipocytes
Humans
Clonogenic assay
Child
Severe combined immunodeficiency
B-Lymphocytes
Multidisciplinary
Osteoblasts
Stem Cells
Mesenchymal stem cell
Immunologic Deficiency Syndromes
Infant
Cell Differentiation
Mesenchymal Stem Cells
medicine.disease
Immunity, Innate
3. Good health
Toll-Like Receptor 3
Toll-Like Receptor 4
030104 developmental biology
medicine.anatomical_structure
Settore MED/38 - PEDIATRIA GENERALE E SPECIALISTICA
Child, Preschool
Cancer research
Medicine
Female
Bone marrow
Stem cell
Subjects
Details
- ISSN :
- 20452322
- Volume :
- 7
- Issue :
- 1
- Database :
- OpenAIRE
- Journal :
- Scientific reports
- Accession number :
- edsair.doi.dedup.....80cd7f6aee0fb5e9c42578ef688a3ecf