Balloon cells in human cortical dysplasia and tuberous sclerosis: isolation of a pathological progenitor-like cell.
Neural stem cells are present in the human post-natal brain and are important in the development of brain tumours. However, their contribution to non-neoplastic human disease is less clear. We have tested the hypothesis that malformations of cortical development contain abnormal (pathological) stem cells. Such malformations are a major cause of epilepsy. Two of the most common malformations [focal cortical dysplasia (FCD) and cortical tubers] are characterised by the presence of a population of abnormal cells known as balloon cells. The identity of these cells is unknown but one hypothesis is that they are an abnormal stem cell that contributes to the pathogenesis of the malformation. We have characterised in tissue, and isolated in culture, an undifferentiated population of balloon cells from surgical resections of FCD and cortical tubers. We show that beta1-integrin labels a sub-population of balloon cells with a stem cell phenotype and show for the first time that these cells can be isolated in vitro. We have characterised the immunohistochemical, morphological and ultrastructural features of these cells. This is the first isolation of an abnormal cell with features of a progenitor/stem cell from a non-neoplastic disease of the brain.
Yasin
Shireena A
SA
Department of Histopathology, Great Ormond Street Hospital, London, UK.
Latak
Kate
K
Becherini
Francesca
F
Ganapathi
Anita
A
Miller
Khadijah
K
Campos
Oliver
O
Picker
Simon R
SR
Bier
Nelly
N
Smith
Martin
M
Thom
Maria
M
Anderson
Glenn
G
Helen Cross
J
J
Harkness
William
W
Harding
Brian
B
Jacques
Thomas S
TS
D001921:Brain / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D002478:Cells, Cultured; D002540:Cerebral Cortex / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D006801:Humans; D039482:Integrin alpha5 / Q000378:metabolism; D019012:Integrin beta1 / Q000378:metabolism; D007382:Intermediate Filaments / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D054220:Malformations of Cortical Development / Q000378:metabolism / Q000473:pathology*; D008928:Mitochondria / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D009457:Neuroglia / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D009474:Neurons / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D013234:Stem Cells / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure; D014402:Tuberous Sclerosis / Q000378:metabolism / Q000473:pathology*
D039482:Integrin alpha5; D019012:Integrin beta1