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Upregulation of adenosine kinase in Rasmussen encephalitis.
- Source :
-
Journal of neuropathology and experimental neurology [J Neuropathol Exp Neurol] 2013 Nov; Vol. 72 (11), pp. 1000-8. - Publication Year :
- 2013
-
Abstract
- Rasmussen encephalitis (RE) is a rare neurologic disorder of childhood characterized by unihemispheric inflammation, progressive neurologic deficits, and intractable focal epilepsy. The pathogenesis of RE is still enigmatic. Adenosine is a key endogenous signaling molecule with anticonvulsive and anti-inflammatory effects, and our previous work demonstrated that dysfunction of the adenosine kinase (ADK)-adenosine system and astrogliosis are the hallmarks of epilepsy. We hypothesized that the epileptogenic mechanisms underlying RE are related to changes in ADK expression and that those changes might be associated with the development of epilepsy in RE patients. Immunohistochemistry was used to examine the expression of ADK and glial fibrillary acidic protein in surgically resected human epileptic cortical specimens from RE patients (n = 12) and compared with control cortical tissues (n = 6). Adenosine kinase expression using Western blot and enzymatic activity for ADK were assessed in RE versus control samples. Focal astrogliosis and marked expression of ADK were observed in the lesions of RE. Significantly greater ADK expression in RE versus controls was demonstrated by Western blot, and greater enzymatic activity for ADK was demonstrated using an enzyme-coupled bioluminescent assay. These results suggest that upregulation of ADK is a common pathologic hallmark of RE and that ADK might be a target in the treatment of epilepsy associated with RE.
- Subjects :
- Adolescent
Astrocytes metabolism
Astrocytes pathology
Cerebral Cortex pathology
Child
Child, Preschool
Encephalitis complications
Encephalitis pathology
Female
Gliosis complications
Gliosis enzymology
Gliosis pathology
Humans
Male
Adenosine Kinase metabolism
Cerebral Cortex enzymology
Encephalitis enzymology
Up-Regulation
Subjects
Details
- Language :
- English
- ISSN :
- 1554-6578
- Volume :
- 72
- Issue :
- 11
- Database :
- MEDLINE
- Journal :
- Journal of neuropathology and experimental neurology
- Publication Type :
- Academic Journal
- Accession number :
- 24128682
- Full Text :
- https://doi.org/10.1097/01.jnen.0000435369.39388.5c