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: GIFU UNIVERSITY
• 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
• 起止时间: 1994 至 1995
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/en/grant/KAKENHI-PROJECT-06670782/
• 关键词: PEROXISOME; ZELLWEGER SYNDROME; MODEL MOUSE; CHINESE HAMSTER OVERY CELL; CHO細胞; 神経細胞移動障害

(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.

（1）分离小鼠过氧化物酶体组装因子1（PAF-1）基因组DNA，目的是通过基因靶向使过氧化物酶体缺乏模型小鼠。（2）通过功能互补的过氧化物酶体缺乏，分离了大鼠PAF-2 cDNA。突变的中国仓鼠Overy（CHO）细胞，使用瞬态转染Assey。 PAF-2 cDNA补充了来自Zellweger综合征患者的成纤维细胞的过氧化物酶体。此外，我们克隆了人PAF-2 cDNA，并证明了Zellweger患者的PAF-2突变。

项目成果

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

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。”

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”

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。

M.Masuno,N.Shimozawa,et.al.: "Assignment of the human peroxisome assembly factor-1 gene(PXMP3)responsible for Zellweger syndrome to chrcmosome 8q21-1 by fluorescene in situ hybridization" Genomics. 20. 141-142 (1994)

M.Masuno,N.Shimozawa,et.al.：“通过荧光原位杂交将负责 Zellweger 综合征的人过氧化物酶体组装因子 1 基因 (PXMP3) 分配给染色体 8q21-1”基因组学。