Delayed maturation and differentiation of neurons in focal cortical dysplasia with the transmantle sign: analysis of layer-specific marker expression

Transmantle dysplasia is a rare type of focal cortical dysplasia (FCD) characterized by expansion of the cortex from the deep white matter to the surface and in which there is a FCD IIA or IIB pathologic pattern. To characterize possible mechanisms underlying this regional disorder of radial migrating cells, we studied the expression patterns of neocortical layer-specific markers using immunohistochemistry in surgical specimens from 5 FCD IIA and 4 FCD IIB cases in children. All neuronal cells expressed the mature neuron marker MAP2/2B but not the microglia markers Iba-1 and CD68. Some layer-specific markers showed distinct expression patterns. TBR1-positive, SATB2-positive, and FOXP1-positive cells were diffusely distributed in the cortex and/or the white matter. TBR1-positive and FOXP1-positive cells were generally more numerous in FCD IIB than in FCD IIA and were mostly in the cortical molecular and upper layers. FOXP1-, FOXP2-, and CUTL1-positive cells also expressed the immature neuron marker, Nestin/PROX1, whereas TBR1-, CTIP2-, and SATB2-positive cells only expressed MAP2/2B. These data highlight differences between FCD IIB and FCD IIA with more cells having the immature marker in upper layer markers in the former. By analyzing layer-specific marker expression patterns, we identified apparent neuronal maturation differences between FCD IIA and FCD IIB in cases of transmantle dysplasia.;博士(医学)・乙第1312号・平成25年5月29日;開始ページ : 741;終了ページ : 749;PubMed番号 : http://www.ncbi.nlm.nih.gov/pubmed/22805777

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書名 Delayed maturation and differentiation of neurons in focal cortical dysplasia with the transmantle sign: analysis of layer-specific marker expression
著作者等 Goto, Yu-ichi
Itoh, Masayuki
Kaido, Takanobu
Kaneko, Yuu
Kimura, Yukio
Nakagawa, Eiji
Otsuki, Taisuke
Saito, Takashi
Saito, Yuko
Sasaki, Masayuki
Sato, Noriko
Sugai, Kenji
Sukigara, Sayuri
Takahashi, Akio
Sakakibara, Takafumi
書名別名 "Transmantle sign"を示す限局性皮質異形成における神経細胞の成熟と分化の未熟性:層特異的マーカー発現による解析
出版元 Waverly Press
ページ数 v.
大きさ 26 cm
ISSN 00223069
NCID AA00703323
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言語 英語
出版国 アメリカ合衆国
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