Increased levels of glial cell-derived neurotrophic factor in CSF of infants with SMA.
The cause of motor neuron death in spinal muscular atrophy is still debated. In experimental animal models, neurotrophic factors have great potency in supporting motor neuron survival and differentiation, but there are no clinical studies on neurotrophin involvement in disease progression and motor neuron dysfunction. The aim of this study was to investigate the expression of three neurotrophic factors: nerve growth factor, brain-derived neurotrophic factor, and glial cell-derived neurotrophic factor in the cerebrospinal fluid of six infants with spinal muscular atrophy type I and six controls. The levels of neurotrophic factors were measured using an immunoenzymatic assay. A statistically significant increase in glial cell-derived neurotrophic factor levels was observed in patients with spinal muscular atrophy, compared with controls, whereas nerve growth factor and brain-derived neurotrophic factor did not show significant differences between groups. Glial cell-derived neurotrophic factor is one of the most powerful survival factors for spinal motor neurons. The increase of glial cell-derived neurotrophic factor may represent a response to the loss and damage of neuronal cells at the site of spinal lesion and is possibly related to axonal sprouting and synaptic reorganization of the damaged spinal motor neurons.
Chiaretti
Antonio
A
Pediatric Intensive Care Unit, Catholic University School of Medicine, Rome, Italy. achiaretti@yahoo.it
Leoni
Chiara
C
Barone
Giuseppe
G
Genovese
Orazio
O
Brahe
Christina
C
Mariotti
Paolo
P
Conti
Giorgio
G
D019208:Brain-Derived Neurotrophic Factor / Q000134:cerebrospinal fluid; D005260:Female; D051100:Glial Cell Line-Derived Neurotrophic Factor / Q000134:cerebrospinal fluid*; D006801:Humans; D007124:Immunoenzyme Techniques; D007223:Infant; D008297:Male; D020932:Nerve Growth Factor / Q000134:cerebrospinal fluid; D014897:Spinal Muscular Atrophies of Childhood / Q000134:cerebrospinal fluid*
D019208:Brain-Derived Neurotrophic Factor; C495039:GDNF protein, human; D051100:Glial Cell Line-Derived Neurotrophic Factor; D020932:Nerve Growth Factor