Evaluation of neural damage in Duchenne muscular dystrophy patients.
The presence of non-progressive cognitive impairment is recognized as a common feature in a substantial proportion of patients with Duchenne muscular dystrophy (DMD). Concurrently, the amyloid beta peptide (Aβ42) protein has been associated with changes in memory and cognitive functions. Also, it has been shown that different subtypes of neural stem/progenitor cells (CD 34, CD 45, nestin) are involved in the innate repair of plasticity mechanisms by the injured brain, in which Nerve Growth Factor (NGF) acts as chemotactic agents to recruit such cells. Accordingly, the present study investigated levels of CD 34, CD 45, nestin and NGF in an attempt to investigate makers of neural regeneration in DMD. Neural damage was assayed in terms of Aβ42. Results showed that Aβ42 (21.9 ± 6.7 vs. 12.13 ± 4.5) was significantly increased among DMD patients compared to controls. NGF (165.8 ± 72 vs. 89.8 ± 35.9) and mononuclear cells expressing nestin (18.9 ± 6 vs. 9 ± 4), CD 45 (64 ± 5.4 vs. 53.3 ± 5.2) and CD34 (75 ± 6.2 vs. 60 ± 4.8) were significantly increased among DMD patients compared to controls. In conclusion cognitive function decline in DMD patients is associated with increased levels of Aβ42, which is suggested to be the cause of brain damage in such patients. The significant increase plasma NFG and in the number of mononuclear cells bearing CD34, CD45 and nestin indicates that regeneration is an ongoing process in these patients. However, this regeneration cannot counterbalance the damage induced by dystrophine mutation and increased Aβ42.
Department of Pediatrics, Genetic Unit, Faculty of Medicine, Cairo University;
Ekram Abdel
EA
Salam
Department of Pediatrics, Genetic Unit, Faculty of Medicine, Cairo University;
Iman Ehsan
IE
Abdel-Meguid
Department of Pediatrics, Faulty of Medicine, Ain Shams University;
Rania
R
Shatla
National Center for Radiation Research and Technology.
Soheir
S
Korraa
D018952:Antigens, CD34 / Q000378:metabolism*; D016022:Case-Control Studies; D002648:Child; D003072:Cognition Disorders / Q000378:metabolism* / Q000503:physiopathology*; D004797:Enzyme-Linked Immunosorbent Assay; D006801:Humans; D017493:Leukocyte Common Antigens / Q000378:metabolism*; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000378:metabolism* / Q000503:physiopathology*; D020932:Nerve Growth Factor / Q000378:metabolism*; D064231:Nestin / Q000378:metabolism*
D018952:Antigens, CD34; D064231:Nestin; D020932:Nerve Growth Factor; D017493:Leukocyte Common Antigens; C516769:PTPRC protein, human
Duchenne muscular dystrophy; cognitive function; neural damage