WEKO3
アイテム
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CSF GANGLIOSIDE ANALYSIS USING A HIGHLY SENSITIVE ENZYME-IMMUNOSTAINING METHOD IN RETT SYNDROME AND OTHER NEUROLOGIC DISEASES
http://hdl.handle.net/10470/23339
http://hdl.handle.net/10470/233399907e327-b25d-41d4-86ff-128599ea4704
名前 / ファイル | ライセンス | アクション |
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KJ00006025547.pdf (929.8 kB)
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Item type | 学術雑誌論文 / Journal Article(1) | |||||
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公開日 | 2010-08-10 | |||||
タイトル | ||||||
タイトル | CSF GANGLIOSIDE ANALYSIS USING A HIGHLY SENSITIVE ENZYME-IMMUNOSTAINING METHOD IN RETT SYNDROME AND OTHER NEUROLOGIC DISEASES | |||||
言語 | ||||||
言語 | eng | |||||
資源タイプ | ||||||
資源タイプ識別子 | http://purl.org/coar/resource_type/c_6501 | |||||
資源タイプ | journal article | |||||
別タイトル | ||||||
その他のタイトル | 高感度酵素抗体法を用いたRett症候群および他の神経疾患における髄液ガングリオシド分析 | |||||
著者名 |
NOVO, Maria de Lourdes P.
× NOVO, Maria de Lourdes P.× SANAI, Yutaka× KAWASHIMA, Ikuo× SUZUKI, Yoshiyuki× ROSEMBERG, Sergio× MORITA, Mariko× OSAWA, Makiko× FUKUYAMA, Yukio |
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著者別名 | ||||||
姓名 | ノヴォ, マリア デ ルルデス P. | |||||
著者別名 | ||||||
姓名 | 左内, 豊 | |||||
著者別名 | ||||||
姓名 | 川島, 育夫 | |||||
著者別名 | ||||||
姓名 | 鈴木, 義之 | |||||
著者別名 | ||||||
姓名 | ローゼンベルグ, セルジオ | |||||
著者別名 | ||||||
姓名 | 森田, 真理子 | |||||
著者別名 | ||||||
姓名 | 大澤, 真木子 | |||||
著者別名 | ||||||
姓名 | 福山, 幸夫 | |||||
出版者 | ||||||
出版者 | 東京女子医科大学学会 | |||||
受付日付 | ||||||
日付 | 2010-08-10 | |||||
日付タイプ | Created | |||||
ISSN | ||||||
収録物識別子タイプ | ISSN | |||||
収録物識別子 | 0040-9022 | |||||
NCID | ||||||
収録物識別子タイプ | NCID | |||||
収録物識別子 | AN00161368 | |||||
書誌情報 |
東京女子医科大学雑誌 巻 66, 号 3, p. 117-127, 発行日 1996-03-25 |
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著者版フラグ | ||||||
出版タイプ | VoR | |||||
出版タイプResource | http://purl.org/coar/version/c_970fb48d4fbd8a85 | |||||
抄録 | ||||||
内容記述タイプ | Abstract | |||||
内容記述 | 年齢2~10歳のRett症候群患者12名と,同年齢の髄液検査が必要であった他疾患患者19名,および正常対照例7名において,脳脊髄液中の5つの主要なガングリオシドGM1, GD1a, GD1b, GT1bおよびGQ1bを感度の高い酵素抗体法で測定した.我々は,この方法を用いて標準曲線を作成し,これらを測定できた.本法は,高感度な検査法であることを証明した.対象症例における脳脊髄液中の総ガングリオシド量は,脳脊髄液1ml当たり,正常対照例で100~900ng,神経疾患患者で25ngから最大5,000ngの値をとっていた.Rett症候群を含め,どの神経疾患でも特別なパターンは認められなかった.過去にRett症候群においては,あるガングリオシドが大脳および小脳で低下しているという所見の報告があったが,この所見と関連する結果は髄液では得られなかった.また,我々が,髄液における5つの主要なガングリオシドの値をRett症候群患者と正常および疾患対照例と比較した所では,そのデータからRett症候群を予測させるような低下パターンを見出すことはできなかった.但し,12例中5例でGD1aが, 4例でGT1bおよびGQ1bの合計値が正常対照例の下限値よりも低下していた.Rett症候群の独歩可能な患児と不可能な患児における髄液ガングリオシド値の比較検討では差がなく,また異った臨床ステージの患者間の比較検討でも差がなかった.しかしながら,髄膜炎の急性期,熱性けいれんの直後,またRett症候群のけいれんの多い児2名を含み,てんかん患児の一部で髄液中のガングリオシド,主として,GD1b,GT1b値が上昇していた. | |||||
抄録 | ||||||
内容記述タイプ | Abstract | |||||
内容記述 | The cerebrospinal fluid (CSF) gangliosides GM1, GDla, GDlb, GTlb and GQlb were measured using a highly sensitive enzyme-immunostaining technique, in 12 patients with Rett syndrome (RS) ranging in age from 2y to 10y, in age-matched patients with various neurologic diseases (n=19) and in normal control subjects (n=7). The method used proved to be highly sensitive; a standard curve was established and we were able to obtain reliable assay results. Total CSF gangliosides varied widely among normal controls (100~900 ng/ml CSF) as well as in those with neurologic diseases (25 to 5,000 ng/ml CSF). No specific ganglioside pattern was found in any of the disease groups, including RS. Although previous reports have shown reductions in the levels of gangliosides in the RS brain, we found no evidence of a predictable and constant pattern of reduced levels of any of the five major CSF gangliotetraose series gangliosides in this syndrome; however, the amount of GDla was reduced in five of the 12 RS patients, and the amount of GQ1b+GT1b was reduced in four as compared with the minimum value obtained in the control group. Analysis of CSF ganglioside levels in RS patients revealed no differences between ambulant and non-ambulant patients, nor were there any differences among patients in different clinical stages. There was, however, a positive correlation between CSF ganglioside levels, mainly the b-pathway gangliosides, and the presence and frequency of seizures in some patients, including the one with febrile convulsion, those in the acute stage of meningitis, two of the RS patients and some of those with epileptic syndromes. | |||||
著者所属 | ||||||
Department of Pediatrics, Tokyo Women's Medical College | ||||||
著者所属 | ||||||
Department of Biochemical Cell Research, Tokyo Metropolitan Institute of Medical Science | ||||||
著者所属 | ||||||
Department of Tumor Immunology, Tokyo Metropolitan Institute of Medical Science | ||||||
著者所属 | ||||||
Department of Clinical Genetics, Tokyo Metropolitan Institute of Medical Science | ||||||
著者所属 | ||||||
Department of Pediatrics, Child Neurology Division, Santa Casa School of Medicine:Department of Pathology, Neuropathology Division; University of Sao Paulo | ||||||
著者所属 | ||||||
Department of Pediatrics, Tokyo Women's Medical College | ||||||
著者所属 | ||||||
Department of Pediatrics, Tokyo Women's Medical College | ||||||
著者所属 | ||||||
Department of Pediatrics, Tokyo Women's Medical College | ||||||
著者所属 | ||||||
東京女子医科大学小児科学 | ||||||
著者所属 | ||||||
東京都臨床医学総合研究所生命情報研究部門 | ||||||
著者所属 | ||||||
東京都臨床医学総合研究所腫瘍免疫部門 | ||||||
著者所属 | ||||||
東京都臨床医学総合研究所臨床遺伝学研究部門 | ||||||
著者所属 | ||||||
サンタカザ医科大学病院小児神経科:サンパウロ医科大学病理学 | ||||||
著者所属 | ||||||
東京女子医科大学小児科学 | ||||||
著者所属 | ||||||
東京女子医科大学小児科学 | ||||||
著者所属 | ||||||
東京女子医科大学小児科学 |