Blood clotting factor IX niigata: Substitution of alanine-390 by valine in the catalytic domain

Mitsuhiko Sugimoto, Toshiyuki Miyata, Shun-Ichiro Kawabata, Akira Yoshioka, Hiromu Fukui, Hoyo Takahashi, Sadakki Iwanaga

研究成果: ジャーナルへの寄稿記事

13 引用 (Scopus)

抄録

Factor IX Niigata is a mutant factor IX responsible for the moderately severe hemophilia B in a patient who has a normal level of factor IX antigen with reduced clotting activity (1- 4% of normal). We reported previously that the purified mutant protein could be converted to the factor IXaβ form by factor XIa/Ca2+ at a rate similar to that in the case of normal factor IX, but the resulting mutant factor IXaβ could not activate factor X in the presence of factor VIII, Ca2+ and phospholipids (Yoshioka, A. et al. (1986) Thromb. Res. 42, 595-604). In the present study, we analyzed factor IX Niigata at the structural level to elucidate the molecular abnormality responsible for the loss of clotting activity. Amino acid sequence analysis of a peptide obtained on lysyl endopeptidase digestion, coupled with subsequent SP-V8 digestion, demonstrated that the alanine at position 390 was substituted by valine in the catalytic domain of the factor IX Niigata molecule.

元の言語英語
ページ(範囲)878-880
ページ数3
ジャーナルJournal of biochemistry
104
発行部数6
DOI
出版物ステータス出版済み - 1 1 1988

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Factor IX
Blood Coagulation Factors
Valine
Blood Coagulation
Factor IXa
Alanine
Catalytic Domain
Blood
Substitution reactions
lysyl endopeptidase
Digestion
Factor XIa
Hemophilia B
Factor X
Protein Sequence Analysis
Factor VIII
Mutant Proteins
Phospholipids
Antigens
Amino Acids

All Science Journal Classification (ASJC) codes

  • Biochemistry
  • Molecular Biology

これを引用

Blood clotting factor IX niigata : Substitution of alanine-390 by valine in the catalytic domain. / Sugimoto, Mitsuhiko; Miyata, Toshiyuki; Kawabata, Shun-Ichiro; Yoshioka, Akira; Fukui, Hiromu; Takahashi, Hoyo; Iwanaga, Sadakki.

:: Journal of biochemistry, 巻 104, 番号 6, 01.01.1988, p. 878-880.

研究成果: ジャーナルへの寄稿記事

Sugimoto, Mitsuhiko ; Miyata, Toshiyuki ; Kawabata, Shun-Ichiro ; Yoshioka, Akira ; Fukui, Hiromu ; Takahashi, Hoyo ; Iwanaga, Sadakki. / Blood clotting factor IX niigata : Substitution of alanine-390 by valine in the catalytic domain. :: Journal of biochemistry. 1988 ; 巻 104, 番号 6. pp. 878-880.
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abstract = "Factor IX Niigata is a mutant factor IX responsible for the moderately severe hemophilia B in a patient who has a normal level of factor IX antigen with reduced clotting activity (1- 4{\%} of normal). We reported previously that the purified mutant protein could be converted to the factor IXaβ form by factor XIa/Ca2+ at a rate similar to that in the case of normal factor IX, but the resulting mutant factor IXaβ could not activate factor X in the presence of factor VIII, Ca2+ and phospholipids (Yoshioka, A. et al. (1986) Thromb. Res. 42, 595-604). In the present study, we analyzed factor IX Niigata at the structural level to elucidate the molecular abnormality responsible for the loss of clotting activity. Amino acid sequence analysis of a peptide obtained on lysyl endopeptidase digestion, coupled with subsequent SP-V8 digestion, demonstrated that the alanine at position 390 was substituted by valine in the catalytic domain of the factor IX Niigata molecule.",
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AU - Sugimoto, Mitsuhiko

AU - Miyata, Toshiyuki

AU - Kawabata, Shun-Ichiro

AU - Yoshioka, Akira

AU - Fukui, Hiromu

AU - Takahashi, Hoyo

AU - Iwanaga, Sadakki

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N2 - Factor IX Niigata is a mutant factor IX responsible for the moderately severe hemophilia B in a patient who has a normal level of factor IX antigen with reduced clotting activity (1- 4% of normal). We reported previously that the purified mutant protein could be converted to the factor IXaβ form by factor XIa/Ca2+ at a rate similar to that in the case of normal factor IX, but the resulting mutant factor IXaβ could not activate factor X in the presence of factor VIII, Ca2+ and phospholipids (Yoshioka, A. et al. (1986) Thromb. Res. 42, 595-604). In the present study, we analyzed factor IX Niigata at the structural level to elucidate the molecular abnormality responsible for the loss of clotting activity. Amino acid sequence analysis of a peptide obtained on lysyl endopeptidase digestion, coupled with subsequent SP-V8 digestion, demonstrated that the alanine at position 390 was substituted by valine in the catalytic domain of the factor IX Niigata molecule.

AB - Factor IX Niigata is a mutant factor IX responsible for the moderately severe hemophilia B in a patient who has a normal level of factor IX antigen with reduced clotting activity (1- 4% of normal). We reported previously that the purified mutant protein could be converted to the factor IXaβ form by factor XIa/Ca2+ at a rate similar to that in the case of normal factor IX, but the resulting mutant factor IXaβ could not activate factor X in the presence of factor VIII, Ca2+ and phospholipids (Yoshioka, A. et al. (1986) Thromb. Res. 42, 595-604). In the present study, we analyzed factor IX Niigata at the structural level to elucidate the molecular abnormality responsible for the loss of clotting activity. Amino acid sequence analysis of a peptide obtained on lysyl endopeptidase digestion, coupled with subsequent SP-V8 digestion, demonstrated that the alanine at position 390 was substituted by valine in the catalytic domain of the factor IX Niigata molecule.

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