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Citations to this article

Hereditary deficiency of the seventh component of complement.
J T Boyer, … , U R Nilsson, T S Zimmerman
J T Boyer, … , U R Nilsson, T S Zimmerman
Published October 1, 1975
Citation Information: J Clin Invest. 1975;56(4):905-913. https://doi.org/10.1172/JCI108170.
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Research Article

Hereditary deficiency of the seventh component of complement.

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Abstract

Deficiency of the seventh component of complement has been found in the serum of a 42-yr-old Caucasian woman who has Raynaud's phenomenon, sclerodactyly, and telangiectasia. Partial deficiency was found in the serum of the patient's parents and children, indicating a pattern of inheritance of autosomal codominance. Transfusion experiments indicated that exogenous C7 had a 91-h halk-life in the patient. There was no evidence for C7 synthesis after transfusion. No C7 inhibitors were detected in the patient's serum. The patient's serum was found to support the activation of complement by both the classical and properdin pathways to the C7 stage. The addition of C7 to the patient's serum permitted it to support hemolytic reactions initiated by either pathway. No defects could be detected in plasma or whole blood coagulation. The patient's serum was deficient in opsonizing unsensitized yeast particles in serum and in the generation of chemotactic factor by antigen-antibody complexes and endotoxin. Both deficiencies were corrected by the addition of C7. These observations suggest a key role for C7 for in vitro yeast phagocytosis and chemotaxis generation. However, the patient's lack of infections indicates a relatively minor role for C7 in human resistance to infection.

Authors

J T Boyer, E P Gall, M E Norman, U R Nilsson, T S Zimmerman

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Total citations by year

Year: 2023 2018 2016 2012 2010 2009 2006 2001 1996 1995 1994 1991 1990 1989 1988 1987 1986 1984 1983 1982 1981 1980 1979 1978 1977 1976 Total
Citations: 1 1 1 1 1 1 2 1 2 1 1 2 1 3 1 1 1 1 4 2 6 4 5 7 12 6 69
Citation information
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Citations to this article (69)

Title and authors Publication Year
Genetic bases of C7 deficiency: systematic review and report of a novel deletion determining functional hemizygosity.
Balduit A, Bianco AM, Mangogna A, Zicari AM, Leonardi L, Cinicola BL, Capponi M, Tommasini A, Agostinis C, d'Adamo AP, Bulla R
Frontiers in immunology 2023
Complement component 5 does not interfere with physiological hemostasis but is essential for Escherichia coli- induced coagulation accompanied by Toll-like receptor 4: The role of C5, CD14 and TLR4 in TF upregulation
A Landsem, H Fure, JK Ludviksen, D Christiansen, C Lau, M Mathisen, G Bergseth, S Nymo, KT Lappegård, TM Woodruff, T Espevik, TE Mollnes, OL Brekke
Clinical & Experimental Immunology 2018
Textbook of Pediatric Rheumatology
E Silverman, J Buyon, E Jaeggi
Textbook of Pediatric Rheumatology 2016
Clinical Cases in Primary Immunodeficiency Diseases
A Aghamohammadi, N Rezaei
2012
Rook's Textbook of Dermatology
JA McGrath, J Uitto
Rook's Textbook of Dermatology 2010
Abnormal platelet function in C3-deficient mice
FC Gushiken, H Han, J Li, RE Rumbaut, V Afshar-Kharghan
Journal of Thrombosis and Haemostasis 2009
Systemic Lupus Erythematosus
M Petri
JCR: Journal of Clinical Rheumatology 2006
Deficiency of the Seventh Component of Complement in a Taiwanese Boy
YC Chiang, SD Shyur, LH Huang, TC Wen, MT Lin, HC Yang, PH Liang
Journal of the Formosan Medical Association 2006
Inflammation
JA Hunt
Encyclopedia of Materials Science and Technology 2001
Organ-specific contribution to circulating C7 levels by the bone marrow and liver in humans
MA Naughton, MJ Walport, R Würzner, MJ Carter, GJ Alexander, JM Goldman, M Botto
European Journal of Immunology 1996
How partial C7 deficiency with chronic and recurrent bacterial infections can mimic total C7 deficiency: temporary restoration of host C7 levels following plasma transfusion
R Würzner, AE Platonov, VB Beloborodov, AI Pereverzev, IV Vershinina, BA Fernie, MJ Hobart, PJ Lachmann, A Orren
Immunology 1996
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Journal of Clinical Investigation 1995
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AE Platonov, R Würzner, B Beloborodov, AM Jones, DV Troshansky, IV Vershinina, PJ Lachmann, A Orren
Immunology 1994
Functionally active complement proteins C6 and C7 detected in C6- and C7-deficient individuals
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Clinical & Experimental Immunology 1991
Functionally active complement proteins C6 and C7 detected in C6- and C7-deficient individuals.
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Advances in Human Genetics
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1990
Inherited Deficiency of the Seventh Component of Complement: Studies of C7-Consuming Activity
Y Koitabashi, M Ikoma, T Miyahira, T Shibawaka, Y Yamaguchi, A Baba
Pediatrics International 1989
Deficit en C7 et infections successives a Neisseria spp
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Médecine et Maladies Infectieuses 1989
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European Journal of Pediatrics 1989
Clinical Approach to Infection in the Compromised Host
RH Rubin, LS Young
1988
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H Keller, S Löke, GM Hänsch, D Jentschura, H Gerhard, DL Heene
Klinische Wochenschrift 1987
Inherited Deficiency of the Seventh Component of Complement Associated with Meningococcal Meningitis: Lack of Serum Bactericidal Activity against Neisseria meningitidis in a Girl with C7 Deficiency and HLA Studies of a C7-Deficient Japanese Family
T Miyake, K Ohta, J Kawamori, T Hirao, Y Akagaki, S Inai
Microbiology and Immunology 1986
Immunodermatology
RE Schopf, M Jung, B Morsches, K Bork
Immunodermatology 1984
A pedigree of deficiency of the ninth component of complement (C9)
T Kusaba, T Kisu, S Inaba, K Sakai, K Okochi, T Yanase
The Japanese Journal of Human Genetics 1983
Membrane damage by complement
S Bhakdi, J Tranum-Jensen
Biochimica et Biophysica Acta (BBA) - Reviews on Biomembranes 1983
Inherited disorders of complement
LC Guenther
Journal of the American Academy of Dermatology 1983
The Physiology of Hemostasis
D Ogston
1983
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G Tappeinek
International Journal of Dermatology 1982
Complement Deficiency and Disease
JA Charlesworth, BA Pussell
Australian and New Zealand Journal of Medicine 1982
Deficiency of c7 with systemic lupus erythematosus. solubilization of immune complexes in complement-deficient sera
HJ Zeitz, GW Miller, TF Lint, MA Ali, H Gewurz
Arthritis & Rheumatism 1981
Immunogenetics and schizophrenia1
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Psychological Medicine 1981
C7 deficiency, abnormal platelet aggregation, and rheumatoid arthritis
M Alcalay, D Bontoux, A Peltier, MC Vial, JM Vilde, JL Wautier
Arthritis & Rheumatism 1981
Genetics and Biosynthesis of Complement Proteins
HR Colten, CA Alper, FS Rosen
New England Journal of Medicine 1981
Immunological Aspects of Rheumatology
WC Dick
1981
XXXII. Tagung gehalten in Westerland/Sylt vom 16. bis 20. September 1980
E Christophers, M Goos
1981
FULMINANT MENINGOCOCCEMIA IN A CHILD WITH HEREDITARY DEFICIENCY OF THE SEVENTH COMPONENT OF COMPLEMENT AND PROTEINURIA
C Loirat, D Buriot, AP Peltier, P Berche, Y Aujard, C Griscelli, H Mathieu
Acta Paediatrica 1980
Recurrent bacterial meningitis in patients with genetic defects of terminal complement components
MR Haeney, RA Thompson, J Faulkner, P Mackintosh, AP Ball
Clinical & Experimental Immunology 1980
Cutaneous infections and disorders of inflammation
ME Miller
Journal of the American Academy of Dermatology 1980
A familial dysfunction of the eighth component of complement (C8)
F Tedesco, M Bardare, AM Giovanetti, G Sirchia
Clinical Immunology and Immunopathology 1980
Clinical and immunological studies in a case of selective complete C1q deficiency
AI Berkel, M Loos, O Sanal, G Mauff, Y Güngen, U Ors, F Ersoy, O Yegin
Clinical & Experimental Immunology 1979
Use of C6- and C7-deficient human sera in quantitative hemolytic assays for C6 and C7
JD Clough, LR Mansfield
Journal of Immunological Methods 1979
Inflammatory Reaction
HZ Movat
1979
Host defense against Neisseria meningitidis requires a complement-dependent bactericidal activity
A Nicholson, IH Lepow
Science 1979
Immune Deficiency
MD Cooper, AR Lawton, PA Miescher, HJ Mueller-Eberhard
1979
Inherited Deficiency of the Seventh Component of Complement Associated with Nephritis PROPENSITY TO FORMATION OF C5̄6̄ AND RELATED C7-CONSUMING ACTIVITY
GR Nemerow, H Gewurz, SG Osofsky, TF Lint
Journal of Clinical Investigation 1978
Genetics of complement deficiencies associated with lupus-like syndromes
PH Schur
Arthritis & Rheumatism 1978
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Familial deficiency of two subunits of the first component of complement. c1r and c1s associated with a lupus erythematosus-like disease
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Arthritis & Rheumatism 1978
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Arthritis & Rheumatism 1978
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Hypocomplementaemia and Disease
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Scottish Medical Journal 1978
Absence of the Eighth Component of Complement in Association with Systemic Lupus Erythematosus-Like Disease
HE Jasin
Journal of Clinical Investigation 1977
Hereditary C7 deficiency. Diagnosis and HLA studies in a French-Canadian family
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Journal of Clinical Investigation 1977
INHERITED DEFICIENCIES OF COMPLEMENT IN MAN
TL Ray, KD Wuepper
International Journal of Dermatology 1977
Severe systemic lupus erythematosus with nephritis in a boy with deficiency of the fourth component of complement
JG Schaller, BG Gilliland, HD Ochs, JP Leddy, LC Agodoa, SI Rosenfeld
Arthritis & Rheumatism 1977
Clinical conditions associated with defective polymorphonuclear leukocyte chemotaxis
PG Quie, KL Cates
The American Journal of Pathology 1977
Complement and host defense against infection
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The Journal of Pediatrics 1977
Linkage between the Gene (or Genes) Controlling Synthesis of the Fourth Component of Complement and the Major Histocompatibility Complex
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New England Journal of Medicine 1977
Disorders of Leukocyte Chemotaxis
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Pediatric Clinics of North America 1977
The Complement System: A cascade Foreboding and unharnessed Whose waters run deep
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Pediatric Clinics of North America 1977
The Year in Hematology
AS Gordon, R Silber, J LoBue
1977
Biological Amplification Systems in Immunology
NK Day, RA Good
1977
Complement, Rheumatic Diseases and The Major Histocompatibility Complex
S Ruddy
Clinics in rheumatic diseases 1977
Human platelet-initiated formation and uptake of the C5-9 complex of human complement
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Human deficiency of the eighth component of complement. The requirement of C8 for serum Neisseria gonorrhoeae bactericidal activity
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Hereditary deficiency of the fifth component of complement in man. I. Clinical, immunochemical, and family studies
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Journal of Clinical Investigation 1976
Hereditary deficiency of the fifth component of complement in man. II. Biological properties of C5-deficient human serum
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Journal of Clinical Investigation 1976
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OD Ratnoff
Journal of Allergy and Clinical Immunology 1976
The function of the proteins in the complement system
DR Schultz
Trends in Biochemical Sciences 1976

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