Abstract:
:Previous studies showed that Fanconi anemia (FA) murine stem cells have defective reconstitution after bone marrow (BM) transplantation. The mechanism underlying this defect is not known. Here, we report defective homing of FA patient BM progenitors transplanted into mouse models. Using cells from patients carrying mutations in FA complementation group A (FA-A), we show that when transplanted into nonobese diabetic/severe combined immunodeficiency (NOD/SCID) recipient mice, FA-A BM cells exhibited impaired homing activity. FA-A cells also showed defects in both cell-cell and cell-matrix adhesion. Complementation of FA-A deficiency by reexpression of FANCA readily restored adhesion of FA-A cells. A significant decrease in the activity of the Rho GTPase Cdc42 was found associated with these defective functions in patient-derived cells, and expression of a constitutively active Cdc42 mutant was able to rescue the adhesion defect of FA-A cells. These results provide the first evidence that FA proteins influence human BM progenitor homing and adhesion via the small GTPase Cdc42-regulated signaling pathway.
journal_name
Bloodjournal_title
Bloodauthors
Zhang X,Shang X,Guo F,Murphy K,Kirby M,Kelly P,Reeves L,Smith FO,Williams DA,Zheng Y,Pang Qdoi
10.1182/blood-2008-03-147090subject
Has Abstractpub_date
2008-09-01 00:00:00pages
1683-6issue
5eissn
0006-4971issn
1528-0020pii
blood-2008-03-147090journal_volume
112pub_type
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