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MOLECULAR ANALYSIS OF PEROXISOME BIOGENESIS DISORDERS

MOLECULAR ANALYSIS OF PEROXISOME BIOGENESIS DISORDERS
过氧化物酶体生物发生紊乱的分子分析
批准号:
06670782
负责人:
SHIMOZAWA Nobuyuki
金额:
$1.09万
依托单位:
依托单位国家:
日本
项目类别:
Grant-in-Aid for General Scientific Research (C)
财政年份:
1994
资助国家:
日本
项目状态:
已结题
起止时间:
1994 至 1995

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中文摘要
翻译
(1)利用基因打靶技术提取小鼠过氧化物酶体组装因子-1(PAF-1)基因组DNA,建立过氧酶体缺乏症模型小鼠。(2)利用瞬时转染法对中国仓鼠卵巢(CHO)细胞的过氧酶体缺陷进行功能互补,获得大鼠PAF-2基因。PAF-2基因可补充Zellweger综合征患者成纤维细胞中的过氧化物体。此外,我们还克隆了人PAF-2基因,并证实了Zellweger患者的PAF-2基因突变。
英文摘要
(1) Mouse peroxisome assembly factor-1 (PAF-1) genomic DNA was isolated for the purpose of making a peroxisome deficient model mouse by gene targetting.(2) Rat PAF-2 cDNA was isolated by functional complementation of peroxisome deficiency of a mutant chinese hamster overy (CHO) cell, using transient transfection assey. PAF-2 cDNA complemented the peroxisomes of the fibroblasts derived from patients with Zellweger syndrome. Furthermore, we cloned human PAF-2 cDNA,and demonstrated the mutations of PAF-2 in the Zellweger patients.
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Toshiro Tsukamoto, Satoshi Miura, Toshiki Nakai, Sadaki Yokota, Nobuyuki Shimozawa, Yasuyuki Suzuki, Tadao Orii, Yukio Fujiki, Fumie Sakai, Akemi Bogaki, Hiroaki Yasumo, Takashi Osumi: "Peroxisome assembly factor-2 ; a putative ATPase cloned by functional
Toshiro Tsukamoto、Satoshi Miura、Toshiki Nakai、Sadaki Yokota、Nobuyuki Shimozawa、Yasuyuki Suzuki、Tadao Orii、Yukio Fujiki、Fumie Sakai、Akemi Bogaki、Hiroaki Yasumo、Takashi Osumi:“过氧化物酶体组装因子-2;一种通过功能性克隆的假定 ATP 酶
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通讯作者:
T.Tsukamoto,N.Shimozawa,et al.: "Peroxisome assembly factor-1:Nonsense mutation in a peroxisome-deficient CHO cell mutant and deletion analysis" Molecular and Cellular Biology. 14. 5458-5465 (1994)
T.Tsukamoto、N.Shimozawa 等人:“过氧化物酶体组装因子 1:过氧化物酶体缺陷型 CHO 细胞突变体中的无义突变和缺失分析”分子和细胞生物学。
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Toshifumi Aoyama, Kenji Tsushima, Masayoshi Souri, Takehiko Kamijo, Yasuyuki Suzuki, Nobuyuki Shimozawa, Tadao Orii, Takashi Hashimoto: "Molecular cloning and functional expression of a human peroxisomal acyl-coenzyme A oxidase." Biochem Biophys Res Commu
Toshifumi Aoyama、Kenji Tsushima、Masayoshi Souri、Takehiko Kamijo、Yasuyuki Suzuki、Nobuyuki Shimozawa、Tadao Orii、Takashi Hashimoto:“人过氧化物酶体酰基辅酶 A 氧化酶的分子克隆和功能表达。”
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Jun-ichi Asano, Yasuyuki Suzuki, Shigehiro Yajima, Kyoko Inoue, Nobuyuki Shimozawa, Naomi Kondo, Masahiko Murase, Tadao Orii: "Effects of erucic acid therapy on Japanese patients with X-linked adrenoleukodystrophy" Brain & Development. 16. 454-458 (1994)
Jun-ichi Asano、Yasuyuki Suzuki、Shigehiro Yajima、Kyoko Inoue、Nobuyuki Shimozawa、Naomi Kondo、Masahiko Murase、Tadao Orii:“芥酸治疗对日本 X 连锁肾上腺脑白质营养不良患者的影响”
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共 38 条
    Research on elucidation of pathology and drug discovery in peroxisomal diseases using stem cells and diseased model organisms
    • 批准号:
      15K15389
    • 项目类别:
      Grant-in-Aid for Challenging Exploratory Research
    • 资助金额:
      $2.33万
    • 财政年份:
      2015
    • 负责人:
      SHIMOZAWA Nobuyuki
    • 依托单位:
    Development of phenotype predictive diagnosis method and treatment in adrenoleukodystrophy by the methods combines patient resource and disease model
    • 批准号:
      15H04875
    • 项目类别:
      Grant-in-Aid for Scientific Research (B)
    • 资助金额:
      $10.82万
    • 财政年份:
      2015
    • 负责人:
      SHIMOZAWA Nobuyuki
    • 依托单位:
    Clarification of the relation between the pathology of neurometabolic diseases and peroxisomal function
    • 批准号:
      24390261
    • 项目类别:
      Grant-in-Aid for Scientific Research (B)
    • 资助金额:
      $10.98万
    • 财政年份:
      2012
    • 负责人:
      SHIMOZAWA Nobuyuki
    • 依托单位:
    Clarification of the pathology of adrenoleukodystrophy
    • 批准号:
      24659492
    • 项目类别:
      Grant-in-Aid for Challenging Exploratory Research
    • 资助金额:
      $2.41万
    • 财政年份:
      2012
    • 负责人:
      SHIMOZAWA Nobuyuki
    • 依托单位:
    海外基金