Correlation of circulating CD133+ progenitor subclasses with a mild phenotype in Duchenne muscular dystrophy patients.
BACKGROUND
Various prognostic serum and cellular markers have been identified for many diseases, such as cardiovascular diseases and tumor pathologies. Here we assessed whether the levels of certain stem cells may predict the progression of Duchenne muscular dystrophy (DMD).


RESULTS
The levels of several subpopulations of circulating stem cells expressing the CD133 antigen were determined by flow cytometry in 70 DMD patients. The correlation between the levels and clinical status was assessed by statistical analysis. The median (+/-SD) age of the population was 10.66+/-3.81 (range 3 to 20 years). The levels of CD133+CXCR4+CD34- stem cells were significantly higher in DMD patients compared to healthy controls (mean+/-standard deviation: 17.38+/-1.38 vs. 11.0+/-1.70; P = 0.03) with a tendency towards decreased levels in older patients. Moreover, the levels of this subpopulation of cells correlated with the clinical condition. In a subgroup of 19 DMD patients after 24 months of follow-up, increased levels of CD133+CXCR4+CD34- cells was shown to be associated with a phenotype characterised by slower disease progression. The circulating CD133+CXCR4+CD34- cells in patients from different ages did not exhibit significant differences in their myogenic and endothelial in vitro differentiation capacity.


CONCLUSIONS
Our results suggest that levels of CD133+CXCR4+CD34- could function as a new prognostic clinical marker for the progression of DMD.
Marchesi
Chiara
C
Stem cell Laboratory, Department of Neurological Science, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Ospedale Maggiore Policlinico, Centro Dino Ferrari, University of Milan, Milan, Italy.
Belicchi
Marzia
M
Meregalli
Mirella
M
Farini
Andrea
A
Cattaneo
Alessandra
A
Parolini
Daniele
D
Gavina
Manuela
M
Porretti
Laura
L
D'Angelo
Maria Grazia
MG
Bresolin
Nereo
N
Cossu
Giulio
G
Torrente
Yvan
Y
D000071916:AC133 Antigen; D000293:Adolescent; D000328:Adult; D015703:Antigens, CD / Q000097:blood* / Q000145:classification; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D006023:Glycoproteins / Q000097:blood* / Q000145:classification; D006801:Humans; D020388:Muscular Dystrophy, Duchenne / Q000097:blood* / Q000276:immunology; D020411:Oligonucleotide Array Sequence Analysis; D010455:Peptides / Q000097:blood* / Q000145:classification; D010641:Phenotype; D020133:Reverse Transcriptase Polymerase Chain Reaction; D012720:Severity of Illness Index
D000071916:AC133 Antigen; D015703:Antigens, CD; D006023:Glycoproteins; C000606023:PROM1 protein, human; D010455:Peptides