title: Focal cortical dysplasia type II (malformations of cortical development) aberrantly expresses apoptotic proteins.
abstract: bcl-XL, bax, bcl-2, and p53 are apoptotic proteins essential to normal neural development. Aberrant expression of these proteins has been observed in several central nervous system neoplasms. Immunoexpression of these markers is studied in 21 patients with focal cortical dysplasia type II (Taylor-type cortical dysplasia; malformations of cortical development) who had undergone lesionectomy for treatment of pharmacoresistant epilepsy. Paraffin immunohistochemistry using standard methodology was performed on representative sections using antibodies to bcl-XL, bax, bcl-2, and p53. Aberrant expression of bcl-XL, bax, bcl-2, and p53 was observed in the majority of cases, with dysmorphic neurons staining positively for bcl-XL, bax, and bcl-2 in 71%, 76%, and 24% of cases, respectively, and balloon cells staining positively for bcl-XL, bax, and bcl-2 in 89%, 78%, and 17% of cases, respectively. Most cases (86%) showed some expression of p53, with the majority showing expression of p53 most prominently in balloon cells. Previous work has shown gangliogliomas and dysembryoplastic neuroepithelial tumors, both dysplasia-associated neoplasms, to demonstrate aberrant expression of apoptotic markers, suggesting a possible common mechanism of development for these 2 processes in patients in whom they coexist.
authors.affiliation: Department of Pathology and Laboratory Medicine, Neuropathology Section, Cleveland Clinic, Cleveland, OH 44195, USA.
authors.forename: Wendy A
authors.initials: WA
authors.lastname: Chamberlain
authors.forename: Richard A
authors.initials: RA
authors.lastname: Prayson
mesh_terms: D000293:Adolescent; D000328:Adult; D017209:Apoptosis / Q000235:genetics / Q000502:physiology*; D051017:Apoptosis Regulatory Proteins / Q000096:biosynthesis* / Q000235:genetics*; D002648:Child; D002675:Child, Preschool; D004827:Epilepsy / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D005260:Female; D018303:Ganglioglioma / Q000737:chemistry / Q000235:genetics / Q000473:pathology; D015972:Gene Expression Regulation, Neoplastic / Q000502:physiology; D006801:Humans; D007223:Infant; D008297:Male; D054220:Malformations of Cortical Development / Q000145:classification / Q000235:genetics / Q000378:metabolism* / Q000473:pathology*; D008875:Middle Aged; D009423:Nervous System Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000096:biosynthesis / Q000235:genetics; D012189:Retrospective Studies; D016159:Tumor Suppressor Protein p53 / Q000096:biosynthesis / Q000235:genetics; D051028:bcl-2-Associated X Protein / Q000096:biosynthesis / Q000235:genetics; D051020:bcl-X Protein / Q000096:biosynthesis / Q000235:genetics
chemical_list: D051017:Apoptosis Regulatory Proteins; C494826:BAX protein, human; C494811:BCL2L1 protein, human; D019253:Proto-Oncogene Proteins c-bcl-2; D016159:Tumor Suppressor Protein p53; D051028:bcl-2-Associated X Protein; D051020:bcl-X Protein