Source:http://linkedlifedata.com/resource/pubmed/id/19572808
Switch to
| Predicate | Object |
|---|---|
| rdf:type | |
| lifeskim:mentions | |
| pubmed:issue |
2
|
| pubmed:dateCreated |
2010-2-26
|
| pubmed:abstractText |
Fanconi anemia (FA) is a rare condition due to the genetic inactivation of the FA/BRCA pathway. During childhood, most FA patients display progressive bone marrow failure (BMF), the mechanism of which has not been clarified to date. We analyzed BM mesenchymal stem cells (MSCs) from a series of 20 FA patients with BMF (patient median age 12.5 years old, range 7-34). Expression of FANCD2 and sensitivity to mitomycin C, differentiation capacities, and hematopoiesis-supporting abilities, as well as proliferation, cell senescence, and telomere length were assessed. FA MSCs demonstrated hypersensitivity to mitomycin C compared to control MSCs, as expected for FA cells. FA MSCs had normal immunophenotype, support long-term culture of hematopoietic stem cells (HSCs), and display normal differentiation capacities. Telomere loss during cell aging was similar for FA and control MSCs. However, FA MSCs showed reduced long-term proliferation ability, higher stem cell factor and interleukin-6 levels, and increased expression of senescent-associated beta-galactosidase compared to normal MSCs, suggesting a potential role of the BM microenvironment in long-term BMF.
|
| pubmed:language |
eng
|
| pubmed:journal | |
| pubmed:citationSubset |
IM
|
| pubmed:chemical | |
| pubmed:status |
MEDLINE
|
| pubmed:month |
Feb
|
| pubmed:issn |
1557-8534
|
| pubmed:author |
pubmed-author:BaruchelAndéA,
pubmed-author:BenbunanMarcM,
pubmed-author:ChomienneChristineC,
pubmed-author:GluckmanElianeE,
pubmed-author:LargheroJérômeJ,
pubmed-author:LeblancThierryT,
pubmed-author:LecourtSéverineS,
pubmed-author:LerouxGwenaelleG,
pubmed-author:MarolleauJean-PierreJP,
pubmed-author:SociéGerardG,
pubmed-author:SoulierJeanJ,
pubmed-author:TernauxBrigitteB,
pubmed-author:VanneauxValérieV
|
| pubmed:issnType |
Electronic
|
| pubmed:volume |
19
|
| pubmed:owner |
NLM
|
| pubmed:authorsComplete |
Y
|
| pubmed:pagination |
203-8
|
| pubmed:meshHeading |
pubmed-meshheading:19572808-Adolescent,
pubmed-meshheading:19572808-Adult,
pubmed-meshheading:19572808-Bone Marrow,
pubmed-meshheading:19572808-Cell Aging,
pubmed-meshheading:19572808-Cell Differentiation,
pubmed-meshheading:19572808-Cell Proliferation,
pubmed-meshheading:19572808-Cells, Cultured,
pubmed-meshheading:19572808-Child,
pubmed-meshheading:19572808-Cohort Studies,
pubmed-meshheading:19572808-Fanconi Anemia,
pubmed-meshheading:19572808-Fanconi Anemia Complementation Group D2 Protein,
pubmed-meshheading:19572808-Female,
pubmed-meshheading:19572808-Flow Cytometry,
pubmed-meshheading:19572808-Humans,
pubmed-meshheading:19572808-Immunoblotting,
pubmed-meshheading:19572808-Immunophenotyping,
pubmed-meshheading:19572808-Interleukin-6,
pubmed-meshheading:19572808-Male,
pubmed-meshheading:19572808-Mesenchymal Stem Cells,
pubmed-meshheading:19572808-Stem Cell Factor,
pubmed-meshheading:19572808-Telomere,
pubmed-meshheading:19572808-Young Adult
|
| pubmed:year |
2010
|
| pubmed:articleTitle |
Bone marrow microenvironment in fanconi anemia: a prospective functional study in a cohort of fanconi anemia patients.
|
| pubmed:affiliation |
Cell Therapy Unit, Assistance Publique-Hôpitaux de Paris, Saint-Louis Hospital, University Paris, Paris, France.
|
| pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
|