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Volume 70, Issue 1
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Kuniaki NARISAWAArticle type: Article
1996 Volume 70 Issue 1 Pages 1-8
Published: January 25, 1996
Released on J-STAGE: December 26, 2017
JOURNAL FREE ACCESSHolocarboxylase 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.View full abstractDownload PDF (814K) -
Hiroshi TAMAI, Takuji MURATA, Takao MORINOBU, Tomoko KUNO, Masatoshi H ...Article type: Article
1996 Volume 70 Issue 1 Pages 9-14
Published: January 25, 1996
Released on J-STAGE: December 26, 2017
JOURNAL FREE ACCESSTo 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.View full abstractDownload PDF (499K) -
Tsuyoshi NAKAMURA, Naoki HAYASHI, Daiji YOSHIHARA, Yasuyoshi TAKESHITA ...Article type: Article
1996 Volume 70 Issue 1 Pages 15-22
Published: January 25, 1996
Released on J-STAGE: December 26, 2017
JOURNAL FREE ACCESSThe effect of medium-chain triglyceride on absorption of retinyl acetate (A-ace) was investigated in Mann-Williamson rats (MW rats). Bile and pancreatic juice secretions were surgically bypassed into the ileum in the animals used in the present study. MW rats received a single dose of isotopically labeled A-ace ([^3H]A-ace) alone with either MCT or long-chain triglyceride (LCT) liquid diet. Percentage absorption of [^3H]A-ace was considerably higher in MW rats fed MCT diet than LCT diet. Residual [^3H]A-ace in the stomach was lower in rats fed MCT than LCT. These results suggest that feeding of MCT improved the absorption of A-ace in rats with malabsorption and that this mechanism may be due to the rapid digestion and movement of MCT in digestive tracts.View full abstractDownload PDF (823K) -
Tomoko INUBUSHI, Michie SHIKIJI, Koichi ENDO, Hisao KAKEGAWA, Yasuo KI ...Article type: Article
1996 Volume 70 Issue 1 Pages 23-28
Published: January 25, 1996
Released on J-STAGE: December 26, 2017
JOURNAL FREE ACCESSIn 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.View full abstractDownload PDF (693K) -
Article type: Appendix
1996 Volume 70 Issue 1 Pages 29-31
Published: January 25, 1996
Released on J-STAGE: December 26, 2017
JOURNAL FREE ACCESSDownload PDF (216K) -
[in Japanese]Article type: Article
1996 Volume 70 Issue 1 Pages 33-34
Published: January 25, 1996
Released on J-STAGE: December 26, 2017
JOURNAL FREE ACCESSDownload PDF (271K) -
[in Japanese]Article type: Article
1996 Volume 70 Issue 1 Pages 34-36
Published: January 25, 1996
Released on J-STAGE: December 26, 2017
JOURNAL FREE ACCESSDownload PDF (390K) -
[in Japanese]Article type: Article
1996 Volume 70 Issue 1 Pages 36-38
Published: January 25, 1996
Released on J-STAGE: December 26, 2017
JOURNAL FREE ACCESSDownload PDF (294K)
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