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Online ISSN : 2424-080X
Print ISSN : 0006-386X
70 巻, 1 号
選択された号の論文の8件中1~8を表示しています
  • 成澤 邦明
    原稿種別: 本文
    1996 年 70 巻 1 号 p. 1-8
    発行日: 1996/01/25
    公開日: 2017/12/26
    ジャーナル フリー
    Holocarboxylase synthetase (HCS) plays an essential role in biotin utilization in cells and its deficiency causes biotin-responsive multiple carboxylase deficiency in humans. We have clones the human HCS cDNA, which maps to chromosome 21 q 22. 1. Two mutations in the HCS genes of Japanese patients with HCS deficiency have been identified : a transition from T to C which causes an amino acid substitution of proline for leucine at position 237 (L237P) and a single guanine base deletion (ΔG 1067) followed by premature termination. Transient expression in cultured fibroblasts from a patient after site-directed mutagenesis showed that the L237P mutation was responsible for decreased HCS activity. Hybridization analysis using allele specific oligonucleotide probes demonstrated that the prevalence of the mutations-L237P and ΔG 1067-was 50% and 30%, respectively, among Japanese patients with HCS deficiency. Methylmalonyl CoA mutase (MCM) is an adenosylcobalamin dependent enzyme. Deficiency of the MCM apoenzyme causes an autosomal recessive disorder known as methylmalonic acidemia (MMA). Five of eight Japanese patients with MMA expressed remarkably decreased amount of MCMmRNA which could not be detected by Northern blot analysis. We were able to amplify and quantify the trace amount of mRNA (0.1-1.0%) by RT-PCR coupled with fluorescent analyzer. The trace amount of MCMmRNA was amplified by nested PCR to obtain sufficient amount of cDNA for sequencing analysis. Sequencing of the amplified cDNA revealed two novel mutations : a nonsense mutation (G to T at nucleotide position 425) and 2bp deletion (ΔCA 769) which resulted in the premature termination by frame shift. The nonsense mutation (G425T) accounts for about 20% of all MMA alleles in the Japanese population.
  • 玉井 浩, 村田 卓士, 森信 孝雄, 久野 友子, 穂積 正俊, 茂地 秀紀, 真砂 光宏, 美濃 真
    原稿種別: 本文
    1996 年 70 巻 1 号 p. 9-14
    発行日: 1996/01/25
    公開日: 2017/12/26
    ジャーナル フリー
    To examine the bioavailability of β-carotene, human young male volunteers were given daily either 60 mg of synthesized all-trans, a naturally-occuring β-carotene preparation derived from Dunaliella bardawil, or a placebo. The all-trans β-carotene levels in the subjects given the synthesized all-trans form were almost twice that for the Dunaliella preparation. The plasma 9-cis levels were found to be higher in the all-trans βcarotene group than those in the Dunaliella group, despite no intake of the 9-cis form in the all-trans group and the higher intake of the 9-cis form in the Dunaliella group. This finding suggests that isomerization of the all-trans form to the 9-cis form may occur in the body either during or after absorption. The CD4/CD8 ratio increased after 44 weeks of βcarotene administration whereas natutal killer cells, memory T cells, and cytotoxic T cells remained unaltered throughout the study.
  • 中村 強, 林 直樹, 吉原 大二, 竹下 保義, 繁田 晴美
    原稿種別: 本文
    1996 年 70 巻 1 号 p. 15-22
    発行日: 1996/01/25
    公開日: 2017/12/26
    ジャーナル フリー
    消化吸収障害を有する患者や消化管術前後の患者へのMCT投与を想定して,A-aceの消化吸収性に及ぼす影響を観察した。すなわち,健常ラットあるいは消化吸収障害ラット(MWラット)にA-aceのラベル化合物([^3H]A-ace)をMCTとともに経口投与した場合の[^3H]A-aceの吸収率に及ぼす影響をサフラワー油(LCT)を対照として比較検討した。さらに,作用機序解明の一環として,消化管内における[^3H]A-aceの移動に及ぼす影響も合わせて比較検討した。その結果,MWラットにおける[^3H]A-aceの吸収率は健常ラットに比べ,有意に抑制されることが認められた。また,MWラットにおける[^3H]A-aceの吸収率はMCTもしくはMCTを含む混合油を投与することで,LCT投与に対し有意に改善させることが認められた。さらに,MCT投与によるA-aceの吸収性に対する改善効果は,胃から小腸へのA-aceの移動を早めることと共に,MCTの優れた消化性に起因することが推察された。
  • 犬伏 知子, 敷地 康恵, 遠藤 晃市, 掛川 寿夫, 岸野 泰雄, 勝沼 信彦
    原稿種別: 本文
    1996 年 70 巻 1 号 p. 23-28
    発行日: 1996/01/25
    公開日: 2017/12/26
    ジャーナル フリー
    In the course of gluconeogenesis from hepatic proteins, the activities of lysosomal cysteine proteinases, such as cathepsins B, L, H and J(C) and deaminating enzymes, such as alanine aminotransferase were changed coordinately. When rats were placed on sufficient starvation, the both activities of cathepsins and aminotransferases in the liver were significantly elevated. Furthermore, the activity of hepatic cathepsin L increased markedly by glucagon treatment and insulin treatment caused marked decrease in the activities of cathepsins L and H. The changes in these cathepsin activities by the starvation or the hormone treatments may resulted in the increase of protein amounts judging from Western blotting analysis. The administration of prednisolone caused marked induction of alanine aminotransferase, however the level of hepatic cathepsins was not changed. The glucocorticoid may participate in the regulation of gluconeogenesis from amino acids digested from dietary proteins.
  • 原稿種別: 付録等
    1996 年 70 巻 1 号 p. 29-31
    発行日: 1996/01/25
    公開日: 2017/12/26
    ジャーナル フリー
  • 笠井 佐夫
    原稿種別: 本文
    1996 年 70 巻 1 号 p. 33-34
    発行日: 1996/01/25
    公開日: 2017/12/26
    ジャーナル フリー
  • 田口 寛
    原稿種別: 本文
    1996 年 70 巻 1 号 p. 34-36
    発行日: 1996/01/25
    公開日: 2017/12/26
    ジャーナル フリー
  • 秋元 健吾
    原稿種別: 本文
    1996 年 70 巻 1 号 p. 36-38
    発行日: 1996/01/25
    公開日: 2017/12/26
    ジャーナル フリー
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