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Volume 73, Issue 10
Displaying 1-18 of 18 articles from this issue
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Kuniaki NARISAWAArticle type: Article
1999 Volume 73 Issue 10 Pages 591-597
Published: October 25, 1999
Released on J-STAGE: December 26, 2017
JOURNAL FREE ACCESSIn inborn errors where the defective enzyme had a cofactor requirement it was found that the administration of large amounts of the vitamin resulted in a clinical and biochemical improvement. A mutation that imposes an exaggerated requirement for a vitamin affects the apoenzyme directly (pyruvate dehydrogenase deficiency, maple syrup urine disease, homocystinuria, etc.), the conver sion of a vitamin into coenzyme (methylmalonic acidemia, vitamin D-dependent rickets type 1, etc.) , or the attachment of vitamin to the apoenzyme (holocarboxylase synthetase (HCS)deficiency). Direct proof has so far been provided that molecular defects in the said gene underlie both the vitamin-responsive and vitaminmonresponsive forms. HCS deficiency is an exception, all HCS-deficient patients being responsive to biotin administration. The mutations identified in the HCS deficient patients were really responsible for their reduced HCS activity. We have proposed the concept that not only the Km of HCS for biotin, but also the V_<max> are important factors in determining the responsiveness to biotin therapy.View full abstractDownload PDF (726K) -
Haruo SHIMADA, Akira MOTOYAMA, Jun KIKUCHI, Ryujiro NAMBAArticle type: Article
1999 Volume 73 Issue 10 Pages 599-606
Published: October 25, 1999
Released on J-STAGE: December 26, 2017
JOURNAL FREE ACCESSNeutral solution of pyridoxine (PN) was degraded under fluorescent lamp (characteristic wave length, 352 nm) in a static condition. Some photolysis products were detected and isolated by HPLC. The major component as analyzed by MS and NMR was found to be 5-methyl-4-hydroxy-2,3-bis (hydroxymethyl)pyridine, an isomer of PN. This was due to the photorearrangement of PN. It is well known that PN forms oligomers under heat (the dimer was found in the photolysis products). However, to date, their exact structures remain ambiguous. The oligomers obtained by autoclaving PN were analysed by MS and NMR to determine their accurate structures as well as the mechanisms by which polymerization occurred.View full abstractDownload PDF (623K) -
[in Japanese]Article type: Article
1999 Volume 73 Issue 10 Pages 607-609
Published: October 25, 1999
Released on J-STAGE: December 26, 2017
JOURNAL FREE ACCESSDownload PDF (359K) -
[in Japanese]Article type: Article
1999 Volume 73 Issue 10 Pages 609-612
Published: October 25, 1999
Released on J-STAGE: December 26, 2017
JOURNAL FREE ACCESSDownload PDF (470K) -
[in Japanese], [in Japanese]Article type: Article
1999 Volume 73 Issue 10 Pages 612-613
Published: October 25, 1999
Released on J-STAGE: December 26, 2017
JOURNAL FREE ACCESSDownload PDF (296K) -
[in Japanese]Article type: Article
1999 Volume 73 Issue 10 Pages 613-614
Published: October 25, 1999
Released on J-STAGE: December 26, 2017
JOURNAL FREE ACCESSDownload PDF (297K) -
[in Japanese]Article type: Article
1999 Volume 73 Issue 10 Pages 615-616
Published: October 25, 1999
Released on J-STAGE: December 26, 2017
JOURNAL FREE ACCESSDownload PDF (195K) -
Article type: Bibliography
1999 Volume 73 Issue 10 Pages 617-620
Published: October 25, 1999
Released on J-STAGE: December 26, 2017
JOURNAL FREE ACCESSDownload PDF (329K) -
[in Japanese], [in Japanese], [in Japanese]Article type: Article
1999 Volume 73 Issue 10 Pages 621-
Published: October 25, 1999
Released on J-STAGE: December 26, 2017
JOURNAL FREE ACCESSDownload PDF (158K) -
[in Japanese]Article type: Article
1999 Volume 73 Issue 10 Pages 622-
Published: October 25, 1999
Released on J-STAGE: December 26, 2017
JOURNAL FREE ACCESSDownload PDF (180K) -
[in Japanese], [in Japanese]Article type: Article
1999 Volume 73 Issue 10 Pages 622-623
Published: October 25, 1999
Released on J-STAGE: December 26, 2017
JOURNAL FREE ACCESSDownload PDF (313K) -
[in Japanese]Article type: Article
1999 Volume 73 Issue 10 Pages 623-624
Published: October 25, 1999
Released on J-STAGE: December 26, 2017
JOURNAL FREE ACCESSDownload PDF (321K) -
[in Japanese], [in Japanese], [in Japanese]Article type: Article
1999 Volume 73 Issue 10 Pages 624-625
Published: October 25, 1999
Released on J-STAGE: December 26, 2017
JOURNAL FREE ACCESSDownload PDF (322K) -
[in Japanese]Article type: Article
1999 Volume 73 Issue 10 Pages 625-626
Published: October 25, 1999
Released on J-STAGE: December 26, 2017
JOURNAL FREE ACCESSDownload PDF (317K) -
[in Japanese]Article type: Article
1999 Volume 73 Issue 10 Pages 626-627
Published: October 25, 1999
Released on J-STAGE: December 26, 2017
JOURNAL FREE ACCESSDownload PDF (311K) -
[in Japanese]Article type: Article
1999 Volume 73 Issue 10 Pages 627-
Published: October 25, 1999
Released on J-STAGE: December 26, 2017
JOURNAL FREE ACCESSDownload PDF (180K) -
[in Japanese]Article type: Article
1999 Volume 73 Issue 10 Pages 627-628
Published: October 25, 1999
Released on J-STAGE: December 26, 2017
JOURNAL FREE ACCESSDownload PDF (318K) -
[in Japanese]Article type: Article
1999 Volume 73 Issue 10 Pages 628-629
Published: October 25, 1999
Released on J-STAGE: December 26, 2017
JOURNAL FREE ACCESSDownload PDF (215K)
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