Markers of cellular proliferation are expressed in cortical tubers.
p34cdc2, collapsin response mediator protein 4 (CRMP4), doublecortin (DCX), HuD, and NeuN expression was assessed in tuber (n = 16) and subependymal giant cell astrocytoma (SEGA; n = 6) specimens in tuberous sclerosis complex to define the developmental phenotype and lineage of giant cells (CGs) in these lesions. Many GCs exhibited HuD and NeuN immunolabeling suggesting a differentiated neural phenotype. Giant cells in tubers, SEGAs and subependymal nodules in the Eker rat model of TSC expressed CRMP4 and DCX. Tubers and SEGAs exhibit a heterogeneous profile of differentiation and may share a common cellular lineage. Tubers may contain a subpopulation of newly generated cells.
Department of Neurology, University of Pennsylvania Medical Center, Philadelphia, PA 19104, USA.
Allana
A
Lee
Michelle
M
Maldonado
Marianna
M
Baybis
Christopher A
CA
Walsh
Bernd
B
Scheithauer
Raymond
R
Yeung
Jack
J
Parent
Howard L
HL
Weiner
Peter B
PB
Crino
D015153:Blotting, Western; D002465:Cell Movement / Q000502:physiology*; D002540:Cerebral Cortex / Q000002:abnormalities* / Q000378:metabolism / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D015342:DNA Probes / Q000235:genetics; D018076:DNA, Complementary / Q000235:genetics; D015252:Deoxyribonucleases, Type II Site-Specific / Q000235:genetics* / Q000378:metabolism; D000089662:Doublecortin Protein; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D008297:Male; D017354:Point Mutation / Q000235:genetics; D011506:Proteins / Q000235:genetics* / Q000378:metabolism; D012333:RNA, Messenger / Q000235:genetics; D012097:Repressor Proteins / Q000235:genetics* / Q000378:metabolism; D014402:Tuberous Sclerosis / Q000235:genetics* / Q000378:metabolism / Q000473:pathology*; D000077004:Tuberous Sclerosis Complex 1 Protein; D000077005:Tuberous Sclerosis Complex 2 Protein; D025521:Tumor Suppressor Proteins; D014764:Viral Proteins / Q000235:genetics* / Q000378:metabolism
D015342:DNA Probes; D018076:DNA, Complementary; C000714848:Dcx protein, rat; D000089662:Doublecortin Protein; D011506:Proteins; D012333:RNA, Messenger; D012097:Repressor Proteins; D000077004:Tuberous Sclerosis Complex 1 Protein; D000077005:Tuberous Sclerosis Complex 2 Protein; D025521:Tumor Suppressor Proteins; D014764:Viral Proteins; C090094:SegA protein, Enterobacteria phage T4; D015252:Deoxyribonucleases, Type II Site-Specific