A proteomic analysis of pediatric seizure cases associated with astrocytic inclusions.
Cerebral hyaline astrocytic inclusions have been observed in a subset of patients with early onset epilepsy, brain structural anomalies, and developmental delay, which indicates that it may represent a unique clinicopathologic entity. To further characterize this condition we use proteomics to investigate differentially expressed proteins in epileptic brain tissue from three pediatric epileptic patients with cerebral hyaline astrocytic inclusions, ranging in age from 5-13 years, and compare to brain tissue from two normal controls. Catalase and carbonic anhydrase I both exhibited increased expression in epileptic brain tissue compared to controls. These findings were confirmed by Western blot analysis. Furthermore, both proteins were localized to astrocytes and in epileptic brain were located within the cerebral hyaline astrocytic inclusions, suggesting a potential role in the generation of this pathologic feature of early onset epilepsy with cerebral hyaline astrocytic inclusions.
Tanz Center for Research in Neurodegenerative Diseases, University of Toronto, Toronto, Ontario, Canada.
Naomi P
NP
Visanji
Janice C
JC
Wong
Simon X
SX
Wang
Blair
B
Cappel
Bette K
BK
Kleinschmidt-Demasters
Michael H
MH
Handler
Ayako
A
Ochi
Hiroshi
H
Otsubo
James T
JT
Rutka
Cristina
C
Go
Shelly
S
Weiss
Harry V
HV
Vinters
Cynthia E
CE
Hawkins
Leroi V
LV
Desouza
K W Michael
KW
Siu
Lili-Naz
LN
Hazrati
D000293:Adolescent; D001253:Astrocytes / Q000378:metabolism / Q000473:pathology*; D002256:Carbonic Anhydrases / Q000378:metabolism; D002374:Catalase / Q000378:metabolism; D002675:Child, Preschool; D004827:Epilepsy / Q000175:diagnosis / Q000378:metabolism* / Q000473:pathology*; D005260:Female; D006801:Humans; D006818:Hyalin / Q000737:chemistry; D002479:Inclusion Bodies / Q000378:metabolism / Q000473:pathology*; D040901:Proteomics / Q000379:methods*
D002374:Catalase; D002256:Carbonic Anhydrases