書籍詳細

第1章 血友病の歴史

P.5 掲載の参考文献

• 1) Rosner F:Hemophilia in the Talmud and Rabbinic writings. Ann Intern Med 70:833-837, 1969
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• 2) 弘田長:出血病患者の実験. 東京医学会雑誌3:357-361, 1889

• 3) 山下辨治郎:血友病の一例. 成医会月報243:13-18, 1902

• 4) Brinkhous KM, Hemker HC:A short history of hemophilia, with some comments on the word "Hemophilia" . Handbook of Hemophilia, part 1, Amsterdam:Excerpta Medica, 1975. p 3-20

• 5) 福井弘:血液凝固障害. 新内科学大系50B血液 造血器疾患IIb. 中山書店, 1978. p 151-190

• 6) 福武勝博:血友病の歴史, 血友病全国ヘモフィリア友の会, 1981. p1-17

• 7) Duckert F, Koller F:The old Swiss haemophilia families of Tenna and Wald. Handbook of Hemophilia, part 1, Amsterdam:Excerpta Medica, 1975. p 21-29

• 8) Aggeler PM, et al:Plasma thromboplastin component (PTC) deficiency:a new disease resembling hemophilia. Proc Soc Exp Biol Med 79:692-694, 1952
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• 9) Biggs R, et al:Christmas disease;a condition previously mistaken for haemophilia. Brit Med J 2:1378-1382, 1952
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• 10) Biggs R, Macfarlane RG:Defects in the intrinsic prothrombin activator system including haemophilia, Christmas disease and deficiency of Hageman and PTA factors. Human Blood Coagulation and Its Disorders, 3rd ed, Oxford:Blackwell Scientific Publications, 1962. p 217-256

• 11) 吉田邦男, 他:Plasma thromboplastin component欠乏症による出血性素質. 日本血液学会雑誌 19:511-518, 1956

• 12) Fay PL, et al:Nonenzymatic cofactors:Factor VIII. Amsterdam, New York, Basel:Blood Coagulation Elsevier. 1989. p 35-57

• 13) Vehar GA, Eaton DL:Factor VIII structure and function. Coagulation and Bleeding Disorders, New York, Basel:Marcel Dekker Inc, 1989. p1-21

• 14) Gitschier J:The molecular genetics of hemophilia A. Coagulation and Bleeding Disorders, New York, Basel:Marcel Dekker Inc, 1989. p 23-46

• 15) 西村拓也, 福井弘:血友病の遺伝形式と遺伝診断. 綜合臨牀 38:1773-1780, 1989

• 16) 福井弘, 他:出血時間延長を伴うAHF欠乏症の女児2症例. 日本血液学会雑誌 21:608-614, 1958

P.16 掲載の参考文献

• 1) Yoshida K:Hemophilia and related diseases. Acta Haem Jap 24:109-140, 1961

• 2) Ikkala E:Haemophilia. A study of its laboratory, clinical genetic and social aspects based on knownhaemophiliacs in Finland. Scand J Clin Lab Invest suppl 12:46, 1960

• 3) Sjolin K-E:Haemophilic diseases in Denmark. Oxford:Blackwell Scientific Publications. 1960

• 4) Rangren O:Haemophilia in Sweden. V. Medicosocial aspects. Acta Med Scand 171 suppl:37-60, 179, 1962

• 5) Lurie A, et al:The incidence of haemophilia in South Africa. Handbook of hemophilia, Part I. Amsterdam:Excerpta Medica, 1975. p 49-58

• 6) Mannucci PM, et al:Hemophilia care in Italy. Thrombos Haemostas 35:531-536, 1976

• 7) Mandalaki T:Management of haemophilia in Greece. Thrombos Haemostas 35:522-530, 1976

• 8) Martin-Villar J, et al:Management of hemophilia in Spain. Thrombos Haemostas 35:537-543, 1976

• 9) Allain JP:Management of hemophilia in France. Thrombos Haemostas 35:553-558, 1976

• 10) Brackmann HH, et al:Home care of hemophilia in West Germany. Thrombos Haemostas 35:544-552, 1976

• 11) Rizza CR:The management of haemophilia in the United Kingdom. Thrombos Haemostas 35:559-565, 1976

• 12) Graham JB:Population genetics and genetic counseling in hemophilia "A" (classic hemophilia) . Proc XIth Cong WFH, 1976. p1-10

• 13) Eyster ME, et al:The Pennsylvania hemophilia program 1973-1978. Am J Hemat 9:277-286, 1980
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• 14) Larsson SA, et al:Current status of Swedish hemophiliacs. Acta Med Scand 212:195-200, 1982
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• 15) Ikkala E, et al:Changes in the life expectancy of patients with severe haemophilia A in Finland in 1930-79. Brit J Haemat 52:7-12, 1982
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• 16) 吉田邦男:血友病の疫学. 血友病, 名古屋:全国ヘモフィリア友の会, 1981. p19-58

P.27 掲載の参考文献

• 1) Kay LA:Essential of Haemostasis and Thrombosis. Edinburgh:Churchill Livingstone, 1988

• 2) Colman R, et al:Hemostasis and Thrombosis:Basic Principles and Clinical Practice. Philadelphia:JB Lippincott, 1987

• 3) Bloom A, et al:Haemostasis and Thrombosis. Edinburgh:Churchill Livingstone, 1987

• 4) Ratnoff OD, et al:Disorders of Haemostasis, 2nd edition. Philadelphia:WB Saunders Co. 1991

• 5) Sixma J, et al:1991 State of the Art. Thrombosis and Haemostasis. No. 1, No. 2, Vol 66. 1991

• 6) Joist J, et al:Current issues in laboratory diagnosis of bleeding disorders and hypercoagulable states. Sem Thromb and Hemost 16:151-204, 1990

• 7) Kwaan HC, et al:clinical aspects of fibrinolysis. Sem Thromb and Hemost 16:207-279, 1990

• 8) 日本臨床病理学会編:凝固・線溶・血小板研究-基礎と臨床. 臨床病理特集第81号, 1989, 第86号, 1990

• 9) 特集:血栓止血の進歩と臨床. 日本臨牀47 (4), 1989

• 10) 山元泰之, 他:血友病患者の血管内皮細胞障害-特に血中トロンボモジュリンとClq結合免疫複合体との関係において-. 東京医科大学雑誌 50:283-289, 1992
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P.37 掲載の参考文献

• 1) 半田 誠, 永井博史:血小板のアゴニストとレセプター(コラゲン) . 血小板 (山中學, 山崎博男, 編), 医学書院, 1991. p47

• 2) 村田 満:血小板GP Ib/IX複合体-vWFとの相互作用の分子機構. 医学のあゆみ 160:677, 1992

• 3) Fujimura Y, Titani K:Structure and function of von Willebrand factor. Hemostasis and Thrombosis, 3rd edition (Bloom AL, ed.), 1992 in press

• 4) Brass LF, et al:GTP-binding proteins and platelet activation. Progress in Hemostasis and Thrombosis (Coller BS, ed.), WB Saunders, 1991. p 127

• 5) Phillips DR, et al:The platelet membrane glycoprotein II b-III a complex. Blood 71:831, 1988

• 6) Bevers EM, et al:Platelets in biology and pathology III (Mac Intyre DE, Gordon JI, eds.), Elsevier, 1987. P127

P.44 掲載の参考文献

• 1) Bloom AL, Thomas DP, eds.:Haematostasis and Thrombosis, second edition, Edinburgh:Churchill Livingstone, 1987

• 2) Halkier T:Mechanisms in Blood Coagulation, Fibrinolysis and the Complement System. Cambridge:Cambridge University Press, 1991

• 3) Patthy L:Evolution of blood coagulation and fibrinolysis. Blood Coag Fibrinol 1:153-166, 1990

• 4) Crook M:Platelet prothrombinase in health and disease. Blood Coag Fibrinol 1:175-182, 1990

• 5) 松田 保:t-PA. 東京:ライフサイエンス, 1991

P.54 掲載の参考文献

• 1) Zimmerman TS, et al:Immunologic differentiation of classical hemophilia (Factor VIII deficiency) andvon Willebrand's disease. J Clin Invest 50:244-254, 1971

• 2) 0wen WG, et al:Antihemophilic factor:Separation of an active fragment following dissociation bysalts or detergents. Thromb Diathes haemorrh 27:502-515, 1972

• 3) Wood WI, et al:Expression of active human factor VIII from recombinant DNA clones. Nature 312:330-337, 1984
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• 4) Norman JC, et al:Antihemophilic factor release perfused liver and spleen. Science 158:1060-1061, 19675) Webster WP, et al:Release of factor VIII (anti-haemophilic factor) from perfused organs and tissues. Nautre 213:1146-1147, 1967

• 6) Lewis JH, et al:Liver transplantation in a hemophiliac. New Engl J Med 312:1189, 1985

• 7) Wion KL, et al:Distribution of factor VIII mRNA and antigens in human liver and other tissues. Nature 317:726-729, 1985
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• 8) Zelechowska MG, et al:Ultrastructural localization of factor VIII procoagulant antigen in human liverhepatocytes. Nature 217:729-730, 1985

• 9) Munro S, et al:An Hsp70-like protein in the ER:Identity with the 78 Kd glucose-regulated protein andimmunoglobulin heavy chain binding protein. Cell 46:291-300, 1986

• 10) Domer AJ, et al:The relationship of N-linked glycosylation and heavy chain-binding protein association with the secretion of glycoproteins. J Cell Biol 105:2665-2674, 1987

• 11) Kaufman RJ, et al:Synthesis processing and secretion of recombinant human factor VIII expressed in mammalian cells. J Biol Chem 263:6352-6362, 1988

• 12) Huttner WB, et al:Protein sulphation of tyrosine. Modem Cell Biol 6:97-140, 1988

• 13) Rotblat F, et al:Purification of human factor VIII:Cand its characterization by western blotting usingmonoclonal antibodies. Biochemistry 24:4292-4300, 1985

• 14) Vehar GA, et al:Structure of human factor VIII. Nature 312:337-342, 1984

• 15) Kane WH, et al:Blood coagulation factor V and VIII:Structural similarities and their relationship tohemorrhagic and thrombotic disorders. Blood 71:539-555, 1988
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• 16) Church WR, et al:Coagulation factor V and VIII and ceruloplasmin constitute a family of structurally related proteins. Proc Natl Acad Sci USA 81:6935-6937, 1984

• 17) Pool S, et al:Sequence and expression of the discoidin I gene family in dictyostelium discoideum. J Mol Biol 153:273-289, 1981

• 18) Eaton DL, et al:Construction and characterization of an active factor VIII variant lacking the central one-third of the molecule. Biochemistry 25:8342-8347, 1986

• 19) Giles AR, et al:A canine model of hemophilic (factor VIII:Cdeficiency) bleeding. Blood 60:727-730, 1982

• 20) Toole JJ, et al:A large region (-95kDa) of human factor VIII is dispensable for in vitro procoagulantactivity. Proc Natl Acad Sci USA 83:5939-5942, 1986

• 21) Vehar GA, et al:Factor VIII structure and function. Coagulation and Bleeding Disorders (Zimmerman TS, et al, eds.), Hematology vol 11, New York:Marcel Dekker, 1989. p1-21

• 22) Koedam JA, et al:Von Willebrand factor protects factor VIII from inactivation by activated protein Cand protein S. Thromb Haemostas 58:223, 1987

• 23) Hamer RJ, et al:The effect of thrombin on the complex between factor VIII and von Willebrand factor. Eur J Biochem 167:253-259, 1987
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• 24) Hill-Eubanks DC, et al:Differential proteolytic activation of factor VIII-von Willebrand factor complexby thrombin. Proc Natl Acad Sci USA 86:6508-6512, 1989

• 25) Lollar P, et al:Stoichiometry of the porcine factor VIII-von Willebrand factor association. J Biol Chem 262:17572-17576, 1987
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• 26) Foster PA, et al:An immunogenic region within residues Val 1670-Glu 16840f the factor VIII light chaininduces antibodies which inhibit binding of factor VIII to von Willebrand factor. J Biol Chem 263:5230-5234, 1988

• 27) Ezban M, et al:Interaction of isolated heavy and light chain of factor VIII:C with the von Willebrandfactor. Research in Clinic and Laboratory 16:112, 1986

• 28) Lollar P, et al:Association of the factor VIII light chain with von Willebrand factor. J Biol Chem 263:10451-10455, 1988
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• 29) Pittman DD, et al:Intemal deletion of factor VIII identify potentially important peptide sequences forbinding to von Willebrand factor. Blood 70:392, 1987

• 30) Sewerin K, et al:The binding between factor VIII and von Willebrand factor. Research in Clinic and Laboratory 16:235, 1986

• 31) O'Brien DP, et al:Purification and characterization of factor VIII 1689 Cys:A non-functional cofactoroccuring in a patient with severe haemophilia A. Blood 73:2117-2122, 1989

• 32) Leyte A, et al:Sulfation of Tyr 1680 of human blood coagulation factor VIII is essential for theinteraction of factor VIII with von Willebrand factor. J Biol Chem 266:740-746, 1991

• 33) Kaufman RJ, et al:The requirement for tyrosine sulfation in factor VIII for functional activity and vWF interaction. Thromb Haemostas 65:817, 1991

• 34) Hamer RJ, et al:The effect of thrombin on the complex between factor VIII and von Willebrand factor. Eur J Biochem 167:253-259, 1987
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• 35) Mosesson MW, et al:Structural model of porcine factor VIII and factor VIIIa molecules based on scanningtransmission electron microscope (STEM) images and STEM mass analysis. J Clin Invest 85:1983-1990, 1990

• 36) Flower WE, et al:Electron microscopy of human factor V and factor VIII:Correlation of morphologywith domain structure and localization of factor V activation fragments. Proc Natl Acad Sci USA 87:7648-7652, 1990

• 37) Fay PJ, et al:Intersubunit fluorescence energy transfer in human factor VIII. J Biol Chem 264:14005-14010, 1989
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• 38) Fay PJ:Reconstitution of human factor VIII from isolated subunits. Arch Biochem Biophys 262:525-531, 1988
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• 39) Lane TM, et al:Ca++dependent structural changes in bovine blood coagulation factor Va and its subunits. Biochemistry 28:4762-4771, 1989

• 40) Fay PJ, et al:Human factor VIIIa subunit structure. Reconstitution of factor VIIIa from the isolated A1/A3-C1-C2 dimer and A2 subunit. J Biol Chem 266:8957-8962, 1991
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• 41) Griffith MJ, et al:Measurement of human factor IXa activity in an isolated factor X activation system. Thromb Res 27:289-301, 1982

• 42) Hultin MB:Role of human factor VIII in factor X activation. J Clin Invest 69:950-958, 1981

• 43) Fulcher CA, et al:Thrombin proteolysis of purified factor VIII procoagulant protein:Correlation ofactivation with generation of a specific polypeptide. Blood 61:807-811, 1983
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• 44) Fulcher CA, et al:Human factor VIII procoagulant protein. Monoclonal antibodies define precursorproduct relationships and functional epitopes. J Clin Invest 76:117-124, 1985
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• 45) Fay PJ:Subunit structure of thrombin activated human factor VIIIa. Biochim Biophys Acta 952:181-190,1987

• 46) Eaton D, et al:Proteolytic processing of human factor VIII:Correlation of specific cleavages by thrombin,
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• 47) Andersson L-O, et al:Isolation and characterization of human factor VIII:Molecular forms in commercial factor VIII concentrate, cryoprecipitate and plasma. Proc Natl Acad Sci USA 83:2979-2983, 1986
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• 48) Pittman DD, et al:Proteolytic requirements for thrombin activation of anti-hemophilic factor (factor VIII) . Proc Natl Acad Sci USA 85:2429-2433, 1988
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• 49) Shima M, et al:An arginine to cysteine amino acid substitution at a critical thrombin cleavage site inadysfunctional factor VIII molecule. Blood 74:1612-1617, 1989

• 50) Hamer RJ, et al:The effect of thrombin on the complex between factor VIII and von Willebrand factor. Eur J Biochem 167:253-259, 1987
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• 51) Lollar P, et al:Subunit structure of thrombin-activated porcine factor VIIL Biochemistry 28:666-674, 1989

• 52) Lollar P, et al:Structural basis for the decreased procoagulant activity of human factor VIII comparedto the porcine homolog. J Biol Chem 266:12481-12486, 1991

• 53) Ware J, et al:Localization of a factor VIII inhibiting antibody epitope to a region between residues 338and 3620f factor VIII heavy chain. Proc Natl Acad Sci USA 85:3165-3169, 1988
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• 54) Foster PA, et al:Localization of the binding regions of a murine monoclonal anti-factor VIII antibodyand a human antifactor VIII allo-antibody, both of which inhibit factor VIII procoagulant activity:to amino acid and residues Thr 351-Ser 3650f the factor VIII heavy chain. J Clin Invest 82:123-128, 1988

• 55) Hemker RJ, et al:The absorption of coagulation factors onto phospholipids. Its role in the reactionmechanisms of blood coagulation. Thromb Diathes haemorrh 24:214-223, 1970

• 56) Lajmanovich A, et al:Human factor VIII procoagulant activity and phospholipid interaction. Biochim Biophys Acta 678:132-136, 1981
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• 57) Yoshioka A, et al:The interaction between factor VIII clotting antigen (VIIICAg) and phospholipid. Brit J Haematol 55:27-36, 1983

• 58) Bloom JW:The interaction of rDNA factor VIII, factor VIII des-797-1562 and factor VIII des-797-1562-derived peptide with phospholipid. Thromb Res 48:439-448, 1987
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• 59) Bartles JR, et al:Discoitin I-membrane interactions. II. Discoitin I binds to and agglutinates negativelycharged phospholipid vesicles. Biochim Biophys Acta 687:129-136, 1982

• 60) Arai M, et al:Molecular basis of factor VIII inhibition by human antibodies. Antibodies that binds to the factor VIII light chain prevent the interaction of factor VIII with phospholipid. J Clin Invest 83:1978-1984, 1989

• 61) Foster PA, et al:Synthetic factor VIII peptides with amino acid sequences contained within the C2 domain of factor VIII inhibit factor VIII binding to phosphatidylserine. Blood 75:1999-2004, 1990
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• 62) Ganz PR, et al:The binding of purified human factor VIII to platelets. Platelet Membrane Receptors, Immunology, Biochemistry and Pathology (Jamison GA, ed.), New York:Allan R. Liss., 1988. p 255-261

• 63) Nesheim ME, et al:The binding of 35S labelled recombinant factor VIII to activated and unactivatedplatelets. J Biol Chem 263:16467-16470, 1988

• 64) Gilbert GE, et al:Platelet-derived microparticles express high affinity receptors for factor VIII. J Biol Chem 266:17261-17268, 1991
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• 65) Bertina RM, et al:Factor IXa protects activated factor VIII against inactivation by activated protein C. Biochim Biophys Res Commun 125:177-183, 1984

• 66) Rick ME, et al:Factor IXa modifies the degradation of factor VIII by activated protein C. Clin Res 36:417, 1988

• 67) Vehar GA, et al:Preparation and properties of bovine factor VIII (antihemophilic factor) . Biochemistry 19:401-410, 1980

• 68) Fulcher CA, et al:Proteolytic inactivation of human factor VIII procoagulant protein by activated humanprotein C and its analogy with factor V. Blood 63:486-489, 1984

• 69) Fay J, et al:Activated protein C-catalyzed inactivation of human factor VIII:Effects of von Willebrand factor and thrombin activation. Thromb Haemostas 65:818, 1991

• 70) Lollar P, et al:Stabilization of thrombin-activated porcine factor VIII:C by factor IXa and phospholipid. Blood 63:1303-1308, 1984
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• 71) Switzer ME, et al:Reactions of thrombin with human factor VIII/von Willebrand factor protein. J Biol Chem 255:10606-10611, 1980

P.67 掲載の参考文献

• 1) Kurachi K, Davie EW:Isolation and characterization of a cDNA coding for human factor IX. Proc Natl Acad Sci USA 79:6461-6464, 1982
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• 2) Yoshitake S, et al:Nucleotide sequence of the gene for human factor IX (Antihemophilic factor B) . Biochemistry 24:3736-3750, 1985
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• 3) Evans JP, et al:Molecular cloning of a cDNA encoding canine factor IX. Blood 74:207-212, 1989
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• 4) Yao S-N, et al:Characterization of a mouse factor IX cDNA and developmental regulation of the factor IX gene expression in liver. Thromb Haemost 65:52-58, 1991
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• 5) Katayama K, et al:Comparison of amino acid sequence of bovine coagulation factor IX (Christmas factor) with that of other vitamin-K dependent plasma proteins. Proc Natl Acad Sci USA 76:4990-4994, 1987

• 6) Thompson AR:Structure, function, and molecular defects of factor IX. Blood 67:565, 1986
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• 7) Salier J-P, et al:Functional characterization of the 5:regulatory region of human factor IX gene. J Biol Chem 265:7062-7068, 1990

• 8) Reijnen MJ, et al:Localization of transcription initiation sites in the human coagulation factor IX gene. FEBS letters 270:207-210, 1990
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• 9) Reitsma PH, et al:Two novel point mutations correlate with an altered developmental expression ofblood coagulation factor IX (Hemophilia B Leyden phenotype) . Blood 73:743-746, 1989

• 10) Jorgensen MJ, et al:Recognition site directing vitamin K-dependent γ-carboxylation residues on thepropeptide of factor IX. Cell 48:185-191, 1987

• 11) Fernlund P, Stenflo J:β-hydroxyaspartic acid in vitamin K-dependent proteins. J Biol Chem 258:12509-12512, 1983

• 12) Hase S, et al:The structure of (Xylose) 2Glucose-o-Serine 53 found in the first epidermal growth factor-like domain of bovine blood clotting factor IX. J Biol Chem 265:1858-1861, 1990

• 13) Ryan J, et al:Structural determinants of the factor IX molecule mediating interaction with the endo thelial cell binding site are distinct from those involved in phospholipid binding. J Biol Chem 264:20283-20287, 1989

• 14) Cheung W-F, et al:The role of the epidermal growth factor-1 and hydrophobic stack domains of humanfactor IX in binding to endothelial cells. J Biol Chem 266:8797-8800, 1991

• 15) Huang LH, et al:Sequence-specific iH NMR assignments, secondary structure, and location of thecalcium binding site in the first epidermal growth factor like domain of blood coagulation factor IX. Biochemistry 30:7402-7409, 1991

• 16) Graham JB, et al:The Malmo polymorphism of factor IX:Establishing the genotypes by rapid analysis of DNA. Blood 73:2104-2107, 1989
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• 17) Thompson AR:Molecular defects in hemophilia B patients. Recombinant Technology in Hemostasis and Thrombosis (Hoyer LW, et al eds.), New York:Plenum Publishing Corp, 1990. p115-131

• 18) Giannelli F, et al:Gene deletions in patients with haemophilia B and anti factor IX antibodies. Nature303:181-182, 1983
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• 19) Tanimoto M, et al:DNA analysis of seven patients with hemophilia B who have anti-factor IXantibodies:relationship to clinical manifestations and evidence that the abnormal gene was inherited. J Lab Clin Med 112:307-313, 1988
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• 20) Green PM, et al:Molecular pathology of haemophilia B. EMBO J 8:1067-1072, 1989
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• 21) Matsushita T, et al:DNA sequence analysis of three inhibitor-positive hemophilia B patients withoutgross gene deletion. Identification of four novel mutations in factor IX gene. J Lab Clin Med 116:492-497, 1990

• 22) Giannelli F, et al:Haemophilia B:database of point mutations and short additions and deletions-secondedition. Nucl Acid Res 19:2193-2219, 1991

• 23) 宮田敏行:第IX因子異常症. 血栓止血誌 2:1-11, 1991

• 24) Wu S-M, et al:In vitroγ-carboxylation of a 59 residue recombinant peptide including the propeptideand theγ-carboxyglutamic acid domain of coagulation factor IX:Effect of mutations near the propeptide cleavage site. J Biol Chem 265:13124-13159, 1990

• 25) McCord DM, et al:Characterization of the functional defect in factor IX Alabama:Evidence for a conformational change due to high affinity calcium binding in the first epidermal growth factor domain. J Biol Chem 265:10250-10254, 1990
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• 26) 西村仁, 他:IX因子Fukuokaの構造および機能異常の解析. 生化学 62:716, 1990

• 27) Bertina RM, et al:Mutation in hemophilia Bm occur at the Arg 180-Val activation site or in the catalytic domain of factor IX. J Biol Chem 265:10876-10883, 1990
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• 28) Bajaj PS, et al:Experimental and theoretical evidence supporting the role of Gly 363 in blood coagula tion factor IXa (Gly 193 in Chymotrypsin) for proper activation of the proenzyme. J Biol Chem 265:2956-2961, 1990

• 29) Miyata Y, et al:Factor IX Amagasaki:A new mutation in the catalytic domain resulting in the loss ofboth coagulant and esterase activities. Biochemistry 30:11286-11291, 1991

• 30) Spitzer SG, et al:Molecular defect in factor IX Bm Lake Elsinore:Substitution of Ala by Val in thecatalytic domain. J Biol Chem 263:10545-10548, 1988

• 31) Sugimoto M, et al:Blood clotting factor IX Niigata:Substitution of alanine-390 by valine in thecatalytic domain. J Biochem 104:878-880, 1988
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• 32) Spizer SG, et al:Replacement of isoleucine-397 by threonine in the clotting proteinase factor IXa (LosAngels and Long Beach variants) affects macromolecular catalysis but not L-tosylarginine methyl esterhydrolysis. Biochem J 265:219-225, 1990

• 33) Bottema CDK, et al:Apast mutation at isoleucine 397 is now a common cause of moderate/mildhaemophilia B. Brit J Haematol 75:212-216, 1990

P.81 掲載の参考文献

• 1) Method for the detection of haemophilia carriers:amemorandum. Bull WHO 55:675-702, 1977
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• 2) 碇久雄:血友病A保因者の第VIII因子. J Nara Med Ass 20:638-654, 1969

• 3) 伊藤多紀:血友病A保因者にたいする運動負荷の影響. 東京医大誌 35:859-871, 1977

• 4) 神谷忠, 他:血友病保因者の診断に対する検討. 日血会誌 39:774-775, 1976

• 5) Rizza CR, et al:Detection of carriers of haemophilia:a "blind" study. Brit J Haematol 30:447-456, 1975

• 6) Lyon MF:Chromosomal and subchromosomal inactivation. Ann Rev Genet 2:31-52, 1968

• 7) Zimmerman TS, et al:Detection of carriers of classical hemophilia using an immunologic assay forantihemophilic factor (factor VIII) . J Clin Invest 50:255-258, 1971

• 8) Ratnoff OD, Jones PK:The laboratory diagnosis of the carrier state for classic hemophilia. Ann Int Med 86:521-528, 1977
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• 9) Ginsburg D, et al:Human von Willebrand Factor (vWF):Isolation of complemental DNA (cDNA) clones and chromosomal localization. Science 228:1401-1406, 1985

• 10) Denson KWE:The detection of factor VIII-like antigen in haemophilic carriers and in patients withraised levels of biologically active factor Vm. Brit J Haematol 24:451-461, 1973

• 11) Ekert H, et al:Detection of carriers of haemophilia. Thromb Diath Haemorrh 30:244-262, 1973.

• 12) Holmberg L, et al:Fifteen-year follow-up of potential carriers of haemophilia A and their progeny. Haemophilia (Ultin ON, Peake IR eds.), New York:American Elsevier Publishing Co., 1975. p47-51

• 13) Panicucci F, et al:Detection of carriers of haemophilia. Haemophilia (Ultin ON, Peake IR eds.), NewYork:American Elsevier Publishing Co., 1975. p 52-55

• 14) Hathaway HS, et al:Carrier detection in classical hemophilia. Pediatrics 57:251-254, 1976
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• 15) Ridgeway H, et al:Detection of the carrier State of hemophlia A in females. Wadley Med Bull 3:68-70, 1973

• 16) Hoyer LW, Rick ME:Implications of immunologic methods for measuring antihemophilic factor (factorVIII) . Ann NY Acad Sci 240:97-108, 1975

• 17) 飯塚敦夫, 他:血友病Aの保因者診断日血会誌39:215, 1976

• 18) Eyster ME, et al:Carrier detection in hemophilia A by combined measurement of immunologic (VIIIAGN) and procoagulant (VIII AHG) activities. Clin Res 23:272A, 1975

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