Abstract
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.
Publication types
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Research Support, Non-U.S. Gov't
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Research Support, U.S. Gov't, P.H.S.
MeSH terms
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Blotting, Western
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Cell Movement / physiology*
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Cerebral Cortex / abnormalities*
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Cerebral Cortex / metabolism
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Cerebral Cortex / pathology*
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Child
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Child, Preschool
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DNA Probes / genetics
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DNA, Complementary / genetics
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Deoxyribonucleases, Type II Site-Specific / genetics*
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Deoxyribonucleases, Type II Site-Specific / metabolism
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Doublecortin Protein
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Female
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Humans
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Immunohistochemistry
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Infant
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Male
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Point Mutation / genetics
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Proteins / genetics*
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Proteins / metabolism
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RNA, Messenger / genetics
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Repressor Proteins / genetics*
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Repressor Proteins / metabolism
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Tuberous Sclerosis / genetics*
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Tuberous Sclerosis / metabolism
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Tuberous Sclerosis / pathology*
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Tuberous Sclerosis Complex 1 Protein
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Tuberous Sclerosis Complex 2 Protein
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Tumor Suppressor Proteins
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Viral Proteins / genetics*
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Viral Proteins / metabolism
Substances
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DNA Probes
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DNA, Complementary
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Dcx protein, rat
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Doublecortin Protein
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Proteins
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RNA, Messenger
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Repressor Proteins
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Tuberous Sclerosis Complex 1 Protein
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Tuberous Sclerosis Complex 2 Protein
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Tumor Suppressor Proteins
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Viral Proteins
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SegA protein, Enterobacteria phage T4
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Deoxyribonucleases, Type II Site-Specific