Nerve growth factor and brain-derived neurotrophic factor in human paediatric hemimegalencephaly.
Paediatric hemimegalencephaly (HME) is a congenital central nervous system (CNS) disorder, characterized by monolateral cerebral hemisphere enlargement, intractable seizures starting in the post-neonatal period, and mental retardation associated with neuropathological anomalies (mainly cortical thickness and lack of lamination). Nerve growth factor (NGF) and brain-derived neurotrophic factor (BDNF) are two neurotrophic factors produced in the mammalian CNS that are involved in the survival, development, and function of a variety of brain cells. In the present study, we found increased cerebral tissue levels of NGF and BDNF in 4 infants with HME; these changes appear to be also associated with abnormal NGF-receptor expression in subcortical blood vessels. Moreover, the marked reduction of cortical choline acetyltransferase immunoreactivity is strongly suggestive of a dysregulation in the NGF differentiative activity in this site that could lead to the pathogenesis of HME.
Institute of Neurobiology and Molecular Medicine, Neurobiology Section CNR, Rome, Italy.
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Antonelli
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Chiaretti
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Amendola
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Piastra
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Di Rocco
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Aloe
D000906:Antibodies / Q000276:immunology; D001921:Brain / Q000002:abnormalities* / Q000276:immunology / Q000473:pathology; D001927:Brain Diseases / Q000235:genetics* / Q000378:metabolism* / Q000473:pathology; D019208:Brain-Derived Neurotrophic Factor / Q000378:metabolism*; D002352:Carrier Proteins / Q000276:immunology; D002675:Child, Preschool; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D007839:Functional Laterality; D006801:Humans; D007223:Infant; D008297:Male; D008565:Membrane Proteins / Q000276:immunology; D020932:Nerve Growth Factor / Q000378:metabolism*; D012333:RNA, Messenger / Q000235:genetics; D020917:Receptor, trkA; D020133:Reverse Transcriptase Polymerase Chain Reaction
D000906:Antibodies; D019208:Brain-Derived Neurotrophic Factor; D002352:Carrier Proteins; D008565:Membrane Proteins; D012333:RNA, Messenger; D020932:Nerve Growth Factor; D020917:Receptor, trkA