title: Characteristic expression of p57/Kip2 in balloon cells in focal cortical dysplasia.
abstract: Balloon cells are a pathognomonic cellular feature of various cortical malformations, including focal cortical dysplasia type IIb (FCD IIb), cortical tubers of tuberous sclerosis (TSC) and hemimegalencephaly (HME). In the present study, we investigated the immunohistochemical expression of p57/Kip2, a member of the Cip/Kip family of cyclin-dependent kinase inhibitory proteins, in balloon cells in surgical specimens taken from 26, 17 and six patients with FCD IIb, TSC and HME, respectively. Characteristic dot-like reactivity with a faint, intense, reticular and process-like pattern was confined to the proximal portion of the cytoplasmic processes of the cells. Immunoelectron microscopy revealed the p57/Kip2 reactivity on intermediate filaments in the proximal portion of the processes. The immunohistochemical profile appeared similar to that of CD34; however, a double immunofluorescence study demonstrated that no cells showed reactivity for both p57/Kip2 and CD34. The frequencies of the p57/Kip2-positive cells in FCD IIb and HME were significantly higher than those in TSC, suggesting that the balloon cells may be heterogeneous. These findings suggest some functional significance of the protein on the cytoplasmic processes of balloon cells and appear consistent with the notion that the cells are abnormally differentiated progenitor cells.
authors.affiliation: Department of Pathology, Brain Research Institute, University of Niigata, Niigata, Japan.|Department of Neurology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan.
authors.forename: Tadashi
authors.initials: T
authors.lastname: Kimura
authors.affiliation: Department of Pathology, Brain Research Institute, University of Niigata, Niigata, Japan.
authors.forename: Hiroki
authors.initials: H
authors.lastname: Kitaura
authors.affiliation: Department of Functional Neurosurgery, Epilepsy Center, Nishi-Niigata Chuo National Hospital, Niigata, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Masuda
authors.affiliation: Department of Functional Neurosurgery, Epilepsy Center, Nishi-Niigata Chuo National Hospital, Niigata, Japan.
authors.forename: Shigeki
authors.initials: S
authors.lastname: Kameyama
authors.affiliation: National Center of Neurology and Psychiatry, Tokyo, Japan.
authors.forename: Yuko
authors.initials: Y
authors.lastname: Saito
authors.affiliation: National Center of Neurology and Psychiatry, Tokyo, Japan.
authors.forename: Kenji
authors.initials: K
authors.lastname: Sugai
authors.affiliation: National Center of Neurology and Psychiatry, Tokyo, Japan.
authors.forename: Taisuke
authors.initials: T
authors.lastname: Otsuki
authors.affiliation: National Medical Center for Children and Mothers, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Atsuko
authors.initials: A
authors.lastname: Nakazawa
authors.affiliation: National Medical Center for Children and Mothers, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Nobuhito
authors.initials: N
authors.lastname: Morota
authors.affiliation: Comprehensive Epilepsy Center, Seirei Hamamatsu General Hospital, Hamamatsu, Japan.
authors.forename: Takamichi
authors.initials: T
authors.lastname: Yamamoto
authors.affiliation: Department of Neurosurgery, University of Hiroshima, Hiroshima, Japan.
authors.forename: Kouji
authors.initials: K
authors.lastname: Iida
authors.affiliation: Department of Neurology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan.
authors.forename: Masanori
authors.initials: M
authors.lastname: Nakagawa
authors.affiliation: Department of Neurology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan.
authors.forename: Toshiki
authors.initials: T
authors.lastname: Mizuno
authors.affiliation: Department of Pathology, Brain Research Institute, University of Niigata, Niigata, Japan.
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Takahashi
authors.affiliation: Department of Pathology, Brain Research Institute, University of Niigata, Niigata, Japan.
authors.forename: Akiyoshi
authors.initials: A
authors.lastname: Kakita
mesh_terms: D000293:Adolescent; D000328:Adult; D002540:Cerebral Cortex / Q000378:metabolism* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D050761:Cyclin-Dependent Kinase Inhibitor p57 / Q000378:metabolism*; D004827:Epilepsy / Q000378:metabolism* / Q000473:pathology; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D065703:Malformations of Cortical Development, Group I / Q000378:metabolism* / Q000473:pathology; D008875:Middle Aged; D009474:Neurons / Q000378:metabolism* / Q000473:pathology; D014402:Tuberous Sclerosis / Q000378:metabolism / Q000473:pathology; D055815:Young Adult
chemical_list: D050761:Cyclin-Dependent Kinase Inhibitor p57
keywords: balloon cell; focal cortical dysplasia; hemimegalencephaly; p57/Kip2; tuberous sclerosis