F S Rosen

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Top papers

Rank Title Journal Year PubWeight™‹?›
1 The enhancement of bacterial phagocytosis by serum. The role of complement components and two cofactors. J Exp Med 1969 4.96
2 Alper CA, Rosen FS: Studies of the in vivo behavior of human C'3 in normal subjects and patients. J Clin Invest 1967 4.45
3 Treatment of hereditary angioedema with a vapor-heated C1 inhibitor concentrate. N Engl J Med 1996 3.89
4 Primary immunodeficiencies. Report of a World Health Organization Committee. Pediatrics 1971 3.69
5 AIDS--an immunologic reevaluation. N Engl J Med 1984 3.53
6 Aberrations of suppressor T cells in human graft-versus-host disease. N Engl J Med 1979 3.10
7 Homozygous deficiency of C3 in a patient with repeated infections. Lancet 1972 3.04
8 The gamma globulins. 3. The antibody deficiency syndromes. N Engl J Med 1966 3.03
9 Studies in vivo and in vitro on an abnormality in the metabolism of C3 in a patient with increased susceptibility to infection. J Clin Invest 1970 2.92
10 Wiskott-Aldrich syndrome protein-deficient mice reveal a role for WASP in T but not B cell activation. Immunity 1998 2.92
11 N-WASP deficiency reveals distinct pathways for cell surface projections and microbial actin-based motility. Nat Cell Biol 2001 2.91
12 Deficiency of C3 inactivator in man. J Immunol 1971 2.86
13 Inactivator of the third component of complement as an inhibitor in the properdin pathway. Proc Natl Acad Sci U S A 1972 2.84
14 Interaction of human thymus-derived and non-thymus-derived lymphocytes in vitro. Induction of proliferation and antibody synthesis in B lymphocytes by a soluble factor released from antigen-stimulated T lymphocytes. J Exp Med 1973 2.75
15 Heterogeneity of "acquired" or common variable agammaglobulinemia. N Engl J Med 1974 2.69
16 Regulation of the B cell response to T-dependent antigens by classical pathway complement. J Immunol 1996 2.51
17 The opsonic fragment of the third component of human complement (C3). J Exp Med 1975 2.51
18 Abnormalities of immunoregulatory T cells in disorders of immune function. N Engl J Med 1979 2.48
19 Deficiency of a granulocyte-membrane glycoprotein (gp150) in a boy with recurrent bacterial infections. N Engl J Med 1982 2.47
20 Implantation of a foetal thymus, restoring immunological competence in a patient with thymic aplasia (Digeorge's syndrome). Lancet 1968 2.40
21 Severe combined immunodeficiency and adenosine deaminase deficiency. N Engl J Med 1975 2.35
22 Hereditary deficiency of the second component of complement (C'2) in man. J Clin Invest 1966 2.32
23 Serum-dependent phagocytosis of paraffin oil emulsified with bacterial lipopolysaccharide. J Exp Med 1973 2.25
24 Cdc42 is required for PIP(2)-induced actin polymerization and early development but not for cell viability. Curr Biol 2000 2.19
25 Hereditary deficiency of the second component of complement (C2) in man: correlation of C2 haemolytic activity with immunochemical measurements of C2 protein. Immunology 1970 2.17
26 Increased susceptibility to infection in a patient with type II essential hypercatabolism of C3. N Engl J Med 1973 2.17
27 Increased susceptibility to infection associated with abnormalities of complement-mediated functions and of the third component of complement (C3). N Engl J Med 1970 2.08
28 The interaction between human monocytes and red cells. Specificity for IgG subclasses and IgG fragments. J Exp Med 1970 2.05
29 Identification and characterization of subpopulations of lymphocytes in human peripheral blood after fractionation on discontinuous gradients of albumin. The cellular defect in X-linked agammaglobulinemia. J Clin Invest 1973 2.05
30 Hereditary deficiency of the second component of complement (C'2) in man: further observations on a second kindred. J Immunol 1967 1.98
31 Persistent and fatal central-nervous-system ECHOvirus infections in patients with agammaglobulinemia. N Engl J Med 1977 1.85
32 Genetically determined heterogeneity of the C1 esterase inhibitor in patients with hereditary angioneurotic edema. J Clin Invest 1971 1.83
33 Selective gamma-g globulin deficiencies in patients with recurrent pyogenic infections. N Engl J Med 1970 1.80
34 Hereditary angioneurotic edema: a clinical survey. Pediatrics 1966 1.75
35 The primary immunodeficiencies (1). N Engl J Med 1984 1.74
36 Molecular characterization of sialophorin (CD43), the lymphocyte surface sialoglycoprotein defective in Wiskott-Aldrich syndrome. Proc Natl Acad Sci U S A 1989 1.73
37 Characterization of a human lymphocyte surface sialoglycoprotein that is defective in Wiskott-Aldrich syndrome. J Exp Med 1984 1.68
38 Expression on blood cells of sialophorin, the surface glycoprotein that is defective in Wiskott-Aldrich syndrome. Blood 1987 1.64
39 Deficiency of suppressor T cells in the hyperimmunoglobulin E syndrome. J Clin Invest 1981 1.63
40 Direct interaction of the Wiskott-Aldrich syndrome protein with the GTPase Cdc42. Proc Natl Acad Sci U S A 1996 1.62
41 Sialophorin, a surface sialoglycoprotein defective in the Wiskott-Aldrich syndrome, is involved in human T lymphocyte proliferation. J Exp Med 1987 1.61
42 CD43, a molecule defective in Wiskott-Aldrich syndrome, binds ICAM-1. Nature 1991 1.60
43 Characterization of human antibody to polyribophosphate, the capsular antigen of Hemophilus influenzae, type B. Clin Immunol Immunopathol 1973 1.60
44 T cells of patients with the Wiskott-Aldrich syndrome have a restricted defect in proliferative responses. J Immunol 1993 1.59
45 Congenital cardiovascular disease and anomalies of the third and fourth pharyngeal pouch. Circulation 1972 1.57
46 Inherited deficiency of the third component of human complement (C'3). J Clin Invest 1969 1.56
47 Pathological events in platelets of Wiskott-Aldrich syndrome patients. Br J Haematol 1999 1.53
48 Morphological abnormalities in the lymphocytes of patients with the Wiskott-Aldrich syndrome. Blood 1986 1.53
49 Endotoxin shock in antibody-deficient mice: unraveling the role of natural antibody and complement in the clearance of lipopolysaccharide. J Immunol 1997 1.52
50 Genetic aspects of the complement system. Adv Immunol 1971 1.52
51 Abnormalities of T cell maturation and regulation in human beings with immunodeficiency disorders. J Clin Invest 1981 1.50
52 Congenital aplasia of the thymus gland (DiGeorge's syndrome). N Engl J Med 1968 1.49
53 Mapping of the X-linked agammaglobulinemia locus by use of restriction fragment-length polymorphism. J Clin Invest 1986 1.49
54 Beta-1C-globulin: metabolism in glomerulonephritis. Science 1966 1.45
55 Reconstitution after transplantation with T-lymphocyte-depleted HLA haplotype-mismatched bone marrow for severe combined immunodeficiency. Proc Natl Acad Sci U S A 1982 1.44
56 Tranexamic acid therapy in hereditary angioneurotic edema. N Engl J Med 1972 1.44
57 The primary immunodeficiencies. (2). N Engl J Med 1984 1.43
58 The mechanism of action of the C3b inactivator (conglutinogen-activating factor) on its naturally occurring substrate, the major fragment of the third component of complement (C3b). J Exp Med 1975 1.43
59 The Wiskott-Aldrich syndrome protein (WASP): roles in signaling and cytoskeletal organization. Annu Rev Immunol 1999 1.42
60 Cellular reactivity to Candida albicans antigen. N Engl J Med 1966 1.41
61 The gamma globulins. 3. The antibody deficiency syndromes. N Engl J Med 1966 1.40
62 Classification of the primary immune deficiencies: WHO recommendation. N Engl J Med 1970 1.39
63 Complete correction of the Wiskott-Aldrich syndrome by allogeneic bone-marrow transplantation. N Engl J Med 1978 1.35
64 In vitro lymphocyte response of patients with immunologic deficiency diseases. N Engl J Med 1970 1.34
65 Heterogeneity of nephritic factor and its identification as an immunoglobulin. Proc Natl Acad Sci U S A 1977 1.34
66 Purification and chemical composition of gpL115, the human lymphocyte surface sialoglycoprotein that is defective in Wiskott-Aldrich syndrome. J Biol Chem 1986 1.33
67 Surface protein abnormalities in lymphocytes and platelets from patients with Wiskott-Aldrich syndrome. Lancet 1982 1.33
68 Gross and ultrastructural observations on lesions produced by intradermal injection of human C3a in man. Am J Pathol 1970 1.32
69 Effect of C'1 esterase on vascular permeability in man: studies in normal and complement-deficient individuals and in patients with hereditary angioneurotic edema. J Clin Invest 1968 1.32
70 Complement deficiencies. Annu Rev Immunol 1992 1.30
71 Opsonic activity in the newborn: role of properdin. Pediatrics 1973 1.30
72 Acquired C1-inhibitor deficiency associated with antiidiotypic antibody to monoclonal immunoglobulins. N Engl J Med 1985 1.30
73 A specific circulating antigen in hamsters infected with Schistosoma mansoni. Detection of antigen in serum and urine, and correlation between antigenic concentration and worm burden. Am J Trop Med Hyg 1969 1.30
74 Classification of primary immunodeficiencies. N Engl J Med 1973 1.28
75 Permeability-increasing activity in hereditary angioneurotic edema plasma. II. Mechanism of formation and partial characterization. J Clin Invest 1969 1.27
76 Chronic neutropenia and abnormal cellular immunity in cartilage-hair hypoplasia. N Engl J Med 1970 1.27
77 Biosynthetic defect in monocytes from human beings with genetic deficiency of the second component of complement. N Engl J Med 1975 1.27
78 Behavior in vivo of normal and dysfunctional C1 inhibitor in normal subjects and patients with hereditary angioneurotic edema. J Clin Invest 1983 1.26
79 Biosynthesis of the third component of complement (C3) in vitro by monocytes from both normal and homozygous C3-deficient humans. J Clin Invest 1977 1.26
80 Bone marrow transplantation only partially restores purine metabolites to normal in adenosine deaminase-deficient patients. J Clin Invest 1981 1.25
81 T cell lines characterize events in the pathogenesis of the Wiskott-Aldrich syndrome. J Exp Med 1992 1.24
82 Deficiency of T helper cells in transient hypogammaglobulinemia of infancy. N Engl J Med 1981 1.23
83 Homozygous human C3 deficiency. The role of C3 in antibody production, C-1s-induced vasopermeability, and cobra venom-induced passive hemolysis. J Clin Invest 1976 1.23
84 Adenosine-deaminase deficiency in a child diagnosed prenatally. Lancet 1975 1.22
85 Polymerase activity in lymphocyte culture supernatants from patients with Kawasaki disease. Nature 1986 1.21
86 Variability in purified dysfunctional C1(-)-inhibitor proteins from patients with hereditary angioneurotic edema. Functional and analytical gel studies. J Clin Invest 1985 1.18
87 Studies on the in vitro behavior of agammaglobulinemic lymphocytes. J Clin Invest 1968 1.17
88 Binding of C3b proceeds by a transesterification reaction at the thiolester site. Nature 1982 1.17
89 Failure of heavy chain glycosylation of IgG in some patients with common, variable agammaglobulinemia. J Clin Invest 1976 1.16
90 Role of the second component of complement (C2) and plasmin in kinin release in hereditary angioneurotic edema (H.A.N.E.) plasma. Trans Assoc Am Physicians 1977 1.16
91 Immunology of human immunodeficiency virus infection and the acquired immunodeficiency syndrome. An update. Ann Intern Med 1987 1.16
92 Nonrandom inactivation of the X chromosome in early lineage hematopoietic cells in carriers of Wiskott-Aldrich syndrome. Blood 1995 1.16
93 A randomized, controlled trial to study the efficacy and safety of C1 inhibitor concentrate in treating hereditary angioedema. Transfusion 1998 1.15
94 Immunodeficiency associated with loss of T4+ inducer T-cell function. N Engl J Med 1981 1.15
95 Inhibition of maturation of human precursor lymphocytes by coformycin, an inhibitor of the enzyme adenosine deaminase. J Exp Med 1976 1.15
96 Characterization of residual enzyme activity in fibroblasts from patients with adenosine deaminase deficiency and combined immunodeficiency: evidence for a mutant enzyme. Proc Natl Acad Sci U S A 1976 1.15
97 Genetics of the complement system. Adv Hum Genet 1976 1.15
98 Further observations on the Swiss type of agammaglobulinemia (alymphocytosis). The effect of syngeneic bone-marrow cells. N Engl J Med 1966 1.15
99 C1 inhibitor: evidence for decreased hepatic synthesis in hereditary angioneurotic edema. Science 1971 1.13
100 Deletions in the ligand for CD40 in X-linked immunoglobulin deficiency with normal or elevated IgM (HIGMX-1). Int Immunol 1993 1.13
101 Enhancement of T-cell activation by the CD43 molecule whose expression is defective in Wiskott-Aldrich syndrome. Nature 1991 1.13
102 Identification of mutations in the Wiskott-Aldrich syndrome gene and characterization of a polymorphic dinucleotide repeat at DXS6940, adjacent to the disease gene. Proc Natl Acad Sci U S A 1995 1.13
103 Early deficit of lymphocytes in Wiskott-Aldrich syndrome: possible role of WASP in human lymphocyte maturation. Clin Exp Immunol 2004 1.13
104 Sialophorin (CD43) and the Wiskott-Aldrich syndrome. Immunodefic Rev 1990 1.12
105 Defects in Wiskott-Aldrich syndrome blood cells. Blood 1996 1.12
106 Unresponsiveness of human B lymphocytes to phytohaemagglutinin. Nature 1974 1.12
107 The gamma globulins. IV. Therapeutic uses of gamma globulin. N Engl J Med 1966 1.12
108 Metabolism of properdin in normal subjects and patients with renal disease. J Clin Invest 1975 1.09
109 Increased serum immunoglobulin E levels following allogeneic bone marrow transplantation. J Allergy Clin Immunol 1980 1.09
110 Establishment of immunological competence in a child with congenital thymic aplasia by a graft of fetal thymus. Lancet 1970 1.08
111 The effect of Fc fragments of IgG on human mononuclear cell responses. Cell Immunol 1980 1.08
112 Somatic mutation of human immunoglobulin V genes in the X-linked HyperIgM syndrome. J Clin Invest 1995 1.08
113 Pernicous anaemia, hypogammaglobulinaemia, and altered lymphocyte reactivity. A family study. Clin Exp Immunol 1972 1.08
114 Primary immunodeficiency. Pediatr Clin North Am 1974 1.08
115 Bone marrow-derived lymphoid cell lines from patients with agammaglobulinemia. J Clin Invest 1978 1.07
116 Hereditary thymic dysplasia: a graft-versus-host reaction induced by bone marrow cells with a partial 4a series histoincompatibility. Pediatr Res 1969 1.07
117 The immunology series comes to an end. N Engl J Med 2001 1.07
118 Localization of the IgG effector site for monocyte receptors. Proc Natl Acad Sci U S A 1975 1.06
119 Hyper immunoglobulin M immunodeficiency. (Dysgammaglobulinemia). Presence of immunoglobulin M-secreting plasmacytoid cells in peripheral blood and failure of immunoglobulin M-immunoglobulin G switch in B-cell differentiation. J Clin Invest 1979 1.06
120 Biosynthesis of human sialophorins and analysis of the polypeptide core. Biochemistry 1987 1.06
121 Dysfunctional C1-inhibitor(At), isolated from a type II hereditary-angio-oedema plasma, contains a P1 'reactive centre' (Arg444----His) mutation. Biochem J 1988 1.06
122 Immunodeficiency to the Epstein-Barr virus in the X-linked recessive lymphoproliferative syndrome. Clin Immunol Immunopathol 1978 1.05
123 Bone-marrow transplantation in severe combined immunodeficiency syndrome. Lancet 1971 1.05
124 Early pre-B cells from normal and X-linked agammaglobulinaemia produce C mu without an attached VH region. Nature 1983 1.05
125 Lymphoma, immunodeficiency and the Epstein-Barr virus. N Engl J Med 1977 1.04
126 A natural model of immunologic tolerance. Tolerance to murine C5 is mediated by T cells, and antigen is required to maintain unresponsiveness. J Exp Med 1982 1.04
127 Purine nucleoside metabolism in the erythrocytes of patients with adenosine deaminase deficiency and severe combined immunodeficiency. J Clin Invest 1976 1.03
128 Human inhibitor of the first component of complement, C1: characterization of cDNA clones and localization of the gene to chromosome 11. Proc Natl Acad Sci U S A 1986 1.03
129 Phenotypic perturbation of B cells in the Wiskott-Aldrich syndrome. Clin Exp Immunol 2005 1.03
130 Increased susceptibility to endotoxin shock in complement C3- and C4-deficient mice is corrected by C1 inhibitor replacement. J Immunol 1997 1.02
131 Genomic organization of the Btk gene and exon scanning for mutations in patients with X-linked agammaglobulinemia. Hum Mol Genet 1994 1.01
132 The acquired immunodeficiency syndrome (AIDS). J Clin Invest 1985 1.00
133 Synthesis of an M component by circulating B lymphocytes in severe combined immunodeficiency. N Engl J Med 1974 1.00
134 The catabolism of C1(-)-inhibitor and the pathogenesis of hereditary angio-edema. Acta Pathol Microbiol Immunol Scand Suppl 1984 1.00
135 Mice naturally tolerant to C5 have T cells that suppress the response to this antigen. Eur J Immunol 1986 0.98
136 Adenosine deaminase activity in normal tissues and tissues from a child with severe combined immunodeficiency and adenosine deaminase deficiency. Clin Immunol Immunopathol 1978 0.98
137 Acquired deficiency of the inhibitor of the first component of complement: report of five additional cases with commentary on the syndrome. J Allergy Clin Immunol 1985 0.97
138 Identification of a subpopulation of lymphocytes in human peripheral blood cytotoxic to autologous fibroblasts. J Exp Med 1976 0.97
139 Angioedema induced by a peptide derived from complement component C2. J Exp Med 1988 0.97
140 Metabolism of gamma globulin fragments in normal and agammaglobulinemic persons. N Engl J Med 1968 0.97
141 Hereditary splenic hypoplasia. Pediatrics 1968 0.97
142 Adenosine deaminase deficiency: frequency and comparative pathology in autosomally recessive severe combined immunodeficiency. Clin Immunol Immunopathol 1979 0.96
143 Interactions of plasma kallikrein and C1-s with normal and dysfunctional C1(-)-inhibitor proteins from patients with hereditary angioneurotic edema: analytic gel studies. Blood 1987 0.96
144 Severe combined immunodeficiency: a pediatric emergency. J Pediatr 1997 0.96
145 The gamma globulins. II. Hypergammaglobulinemia. N Engl J Med 1966 0.95
146 Molecular basis for the deficiency of complement 1 inhibitor in type I hereditary angioneurotic edema. J Clin Invest 1987 0.95
147 Structure of the human sialophorin (CD43) gene. Identification of features atypical of genes encoding integral membrane proteins. Biochem J 1990 0.95
148 Meeting report of the Second International Workshop on Primary Immunodeficiency Disease in Man held in St. Petersburg, Florida, February, 1973. Clin Immunol Immunopathol 1974 0.95
149 Role of complement components in the susceptibility to Plasmodium berghei infection among inbred strains of mice. Ann Trop Med Parasitol 1975 0.94
150 Localization of the gene for the Wiskott-Aldrich syndrome between two flanking markers, TIMP and DXS255, on Xp11.22-Xp11.3. Genomics 1991 0.94
151 Graft-versus-host disease after intrauterine and exchange transfusions for hemolytic disease of the newborn. N Engl J Med 1974 0.94
152 Homozygous C3 deficiency: detection of C3 by radioimmunoassay. Clin Immunol Immunopathol 1977 0.94
153 The lymphocyte and the thymus gland--congenital and hereditary abnormalities. N Engl J Med 1968 0.94
154 An alpha heavy chain abnormality in a child with hypogammaglobulinemia. Clin Immunol Immunopathol 1973 0.93
155 Dysfunctional C1 inhibitor Ta: deletion of Lys-251 results in acquisition of an N-glycosylation site. Proc Natl Acad Sci U S A 1990 0.93
156 Histopathological and ultrastructural observations on tissues from patients with hereditary angioneurotic edema. J Allergy 1971 0.93
157 Transient hypogammaglobulinemia, elevated immunoglobulin E levels, and food allergy. J Allergy Clin Immunol 1979 0.92
158 Discontinuous density gradient analysis of human bone marrow: presence of alloantigen--responsive, PHA--unresponsive cells in norman bone marrow; absence of B lymphocytes in the bone marrow of patients with X--linked agammaglobulinemia. Clin Immunol Immunopathol 1974 0.92
159 The third component of complement: covalent attachment of a radioactive sugar to the labile binding site of C3 via the alternative pathway. J Immunol 1981 0.92
160 Deficiency of Epstein-Barr virus (EBV) receptors on B lymphocytes from certain patients with common varied agammaglobulinemia. J Immunol 1980 0.92
161 WASP and N-WASP in human platelets differ in sensitivity to protease calpain. Blood 2001 0.92
162 A soluble inhibitor of B and T cell proliferation and antibody synthesis produced by dividing human T cells. Cell Immunol 1978 0.92
163 Solubilization of C3 fragments deposited on cross-linked dextran gel beads. Clin Immunol Immunopathol 1979 0.91
164 Transfer of C3 nephritic factor from mother to fetus. Is C3 nephritic factor IgG? N Engl J Med 1977 0.91
165 Disease mechanism: unravelling Wiskott-Aldrich syndrome. Curr Biol 1996 0.91
166 C1s-induced vascular permeability in C2-deficient guinea pigs. J Immunol 1986 0.91
167 WASP levels in platelets and lymphocytes of wiskott-aldrich syndrome patients correlate with cell dysfunction. J Immunol 1999 0.91
168 Immunologic amnesia. Study of an 11-year-old girl with recurrent severe infections associated with dysgammaglobulinemia, lymphopenia and lymphocytotoxic antibody, resulting in loss of immunologic memory. Pediatr Res 1968 0.90
169 Human factor D of the alternative complement pathway. Physicochemical characteristics and N-terminal amino acid sequence. Biochemistry 1979 0.90
170 Reticuloendothelial clearance in cystic fibrosis and other inflammatory lung diseases. N Engl J Med 1988 0.90
171 Detection of genetically determined histocompatibility antigen differences between HL-A identical and MLC nonreactive siblings. Transplantation 1976 0.89
172 Capping and adenosine metabolism. Genetic and pharmacologic studies. J Exp Med 1980 0.89
173 C1 inhibitor hinge region mutations produce dysfunction by different mechanisms. Nat Genet 1992 0.88
174 Safety and efficacy of a monomeric, functionally intact intravenous IgG preparation in patients with primary immunodeficiency syndromes. Clin Immunol Immunopathol 1984 0.88
175 The gamma globulins. I. The structure and synthesis of the immunoglobulins. N Engl J Med 1966 0.88
176 The "neurotic edema" (hereditary angioedema). N Engl J Med 1969 0.87
177 The role of Bruton's tyrosine kinase in B-cell development and function in mice and man. Ann N Y Acad Sci 1995 0.87
178 Recurrent infections, episodic lymphopenia and impaired cellular immunity. N Engl J Med 1969 0.87
179 Restricted classes of immunoglobulin produced by a lymphoid cell line from a patient with agammaglobulinemia. Proc Natl Acad Sci U S A 1978 0.87
180 Studies of a hypomorphic variant of human C3. J Clin Invest 1971 0.86
181 Ontogeny of human T and B lymphocytes during stressed and normal gestation: phenotypic analysis of umbilical cord lymphocytes from term and preterm infants. Clin Immunol Immunopathol 1985 0.86
182 The Xg blood groups and congenital hypogammaglobulinemia. Vox Sang 1966 0.86
183 Immune abnormalities in patients lacking a lymphocyte surface glycoprotein. Clin Immunol Immunopathol 1984 0.86
184 Antihelper T cell autoantibody in acquired agammaglobulinemia. J Clin Invest 1981 0.86
185 Complement: ontogeny and phylogeny. Transplant Proc 1974 0.85
186 Variable expression of WASP in B cell lines of Wiskott-Aldrich syndrome patients. J Immunol 1997 0.85
187 Application of bone marrow transplantation in genetic diseases. Clin Haematol 1983 0.85
188 Scanning of the Wiskott-Aldrich syndrome (WAS) gene: identification of 18 novel alterations including a possible mutation hotspot at Arg86 resulting in thrombocytopenia, a mild WAS phenotype. Hum Mol Genet 1995 0.84
189 Kinin generation in hereditary angioneurotic edema (H.A.N.E.) plasma. Adv Exp Med Biol 1983 0.84
190 Induction of human immunoglobulin synthesis and secretion in somatic cell hybrids of mouse myeloma and human B lymphocytes from patients with agammaglobulinemia. J Exp Med 1978 0.84
191 Transinhibition of C1 inhibitor synthesis in type I hereditary angioneurotic edema. J Clin Invest 1993 0.83
192 Genetic mapping of the Wiskott-Aldrich syndrome with two highly-linked polymorphic DNA markers. Genomics 1988 0.83
193 A cluster of mutations within a short triplet repeat in the C1 inhibitor gene. Proc Natl Acad Sci U S A 1994 0.83
194 Canine pain syndrome is a model for the study of Kawasaki disease. Perspect Biol Med 1991 0.82
195 Molecular defects in hereditary angioneurotic edema. Proc Assoc Am Physicians 1997 0.82
196 Proteolytic fragmentation of sialophorin (CD43). Localization of the activation-inducing site and examination of the role of sialic acid. J Immunol 1990 0.82
197 Structural differences between the two human complement C4 isotypes affect the humoral immune response. J Exp Med 1992 0.82
198 Multiple antigens are altered on T and B lymphocytes from peripheral blood and spleen of patients with Wiskott-Aldrich syndrome. Clin Exp Immunol 1996 0.82
199 X chromosome linked immunodeficiency. Immunodefic Rev 1990 0.82
200 Simultaneous turnover of normal and dysfunctional C1 inhibitor as a probe of in vivo activation of C1 and contact activatable proteases. Clin Exp Immunol 1985 0.81
201 Pathways to the complement system. N Engl J Med 1972 0.81
202 Synthesis of C1 inhibitor in fibroblasts from patients with type I and type II hereditary angioneurotic edema. J Clin Invest 1991 0.81
203 Zoonotic filariasis with lymphedema in an immunodeficient infant. N Engl J Med 1984 0.81
204 A novel protocol to identify mutations in patients with wiskott-Aldrich syndrome. Blood Cells Mol Dis 2002 0.81
205 Physical mapping in a YAC contig of 11 markers on the human X chromosome in Xp11.23. Genomics 1994 0.81
206 The metabolism of C1 inhibitor and C1q in patients with acquired C1-inhibitor deficiency. J Allergy Clin Immunol 1986 0.81
207 The complement system and increased susceptibility to infection. Semin Hematol 1971 0.81
208 Immunodeficiency with defective T-cell response to interleukin 1. Proc Natl Acad Sci U S A 1984 0.81
209 Genetics of complement. Curr Top Hematol 1980 0.81
210 Studies with intravenous gamma globulin. Vox Sang 1967 0.81
211 Characterization of germline mutations of the gene encoding Bruton's tyrosine kinase in families with X-linked agammaglobulinemia. Hum Mutat 1995 0.81
212 Enzyme defects and immune dysfunction. Nature 1978 0.80
213 Transplantation in severe combined immunodeficiency disease with hl-a identical bone marrow. Birth Defects Orig Artic Ser 1975 0.80
214 Tranexamic acid: preoperative prophylactic therapy for patients with hereditary angioneurotic edema. J Allergy Clin Immunol 1977 0.80
215 Inhibition of C1s-induced vascular leakage in guinea pigs by substituted benzamidine and pyridinium compounds. J Immunol 1977 0.79
216 Complement in the serum and venom of Brazilian snakes (Crotalidae). Acta Pathol Microbiol Immunol Scand Suppl 1984 0.79
217 A point mutation in exon 2 of the CD40 ligand gene causes the simultaneous expression of two defective mRNA species in X-linked hyperimmunoglobulinemia M. Hum Mol Genet 1995 0.79
218 Inherited deficiencies of complement proteins in man. Springer Semin Immunopathol 1984 0.79
219 Acquired C1 inhibitor deficiency as a result of an autoantibody to the reactive center region of C1 inhibitor. J Immunol 1994 0.79
220 Clinical applications of complement assays. Adv Intern Med 1975 0.78
221 Isotypes of surface immunoglobulin on B lymphocytes from patients with immune deficiency. J Clin Immunol 1982 0.78
222 Observations on the ultrastructure of lesions induced in human and guinea pig skin by C 1 esterase and polypeptide from hereditary angioneurotic edema (HANE) plasma. Clin Immunol Immunopathol 1975 0.78
223 Defects in cell-mediated immunity. Clin Immunol Immunopathol 1986 0.78
224 New concepts of immunodeficiency. Am J Med 1981 0.77
225 Complement and immunologic disease. Arthritis Rheum 1977 0.77
226 Assessment of the B-lymphocyte population in agammaglobulinemia. Birth Defects Orig Artic Ser 1975 0.77
227 Purification and biochemical characterization of human lymphocyte mitogenic factor (LMF). J Immunol 1979 0.77
228 Gamma-globulin replacement therapy in immunodeficiency. Clin Immunol Immunopathol 1987 0.77
229 The cytoskeletal linker protein moesin: decreased levels in Wiskott-Aldrich syndrome platelets and identification of a cleavage pathway in normal platelets. Br J Haematol 1999 0.77
230 The prognostic value of acute phase reactants in patients with neuroblastoma. Cancer 1977 0.77
231 The genetic basis of immunoglobulin-class switching. N Engl J Med 1994 0.77
232 Localization of the genetic defect in X-linked immunoglobulin deficiency with normal or elevated IgM (HIGMX-1) to the CD40 ligand gene. Trans Assoc Am Physicians 1993 0.76
233 The evolution of MHC restrictions in antigen recognition by T cells in a haploidentical bone marrow transplant recipient. J Immunol 1989 0.76
234 Genetics and biosynthesis of complement proteins. N Engl J Med 1981 0.76
235 First description of an acquired immunodeficiency. Immunodefic Rev 1988 0.75
236 The enigma of the Wiskott-Aldrich syndrome begins to unravel. J Clin Invest 1996 0.75
237 IgM-restricted production of immunoglobulin by lymphoid cell lines from patients with immunodeficiency with hyper IgM (dysgammaglobulinemia). Clin Immunol Immunopathol 1981 0.75
238 Attempted immunologic reconstitution of patients with combined immune deficiency syndrome with bone marrow transplantation from histoincompatible donors. Birth Defects Orig Artic Ser 1975 0.75
239 Latent inherited disease. Am J Med Sci 1968 0.75
240 Androgen therapy in hereditary angioneurotic edema. N Engl J Med 1976 0.75
241 Somatic cell hybrids of mouse myeloma cells and B lymphocytes from a patient with agammaglobulinemia: failure to secrete human immunoglobulin. J Immunol 1979 0.75
242 IgG subclass studies of C3 nephritic factor. Clin Immunol Immunopathol 1978 0.75
243 Neonatal susceptibility to infection. Calif Med 1973 0.75
244 Intravenous dissociated gamma globulin in control of hypogammaglobulinemia. Vox Sang 1969 0.75
245 "Elsewhere in this issue..." [ediotiral]. N Engl J Med 1979 0.75
246 The role of serum factors in the enhancement of phagocytosis. Vox Sang 1969 0.75
247 The gamma globulins. I. The structure and synthesis of the immunoglobulins. N Engl J Med 1966 0.75
248 Attachment of [35S]cysteine to the labile site of the third component of complement via the alternative pathway. Clin Immunol Immunopathol 1983 0.75
249 The gamma globulins. II. Hypergammaglobulinemia. N Engl J Med 1966 0.75
250 The use of cell separation techniques and isantibody to host antigens in the treatent of severe combined immunodeficiency disease with HL-A incompatible maternal marrow. Exp Hematol 1974 0.75
251 The gamma globulin content of meconium and amniotic fluid and the intestinal immunity of the newborn to poliovirus. Arch Gesamte Virusforsch 1967 0.75
252 Sickle-cell disease and the properdin system. N Engl J Med 1973 0.75
253 A change in HL-A phenotype associated with a specific blocking factor in the serum of an infant with severe combined immunodeficiency disease. Transplantation 1972 0.75
254 Hereditary angioneurotic edema and its correction with androgen therapy. Birth Defects Orig Artic Ser 1980 0.75
255 The primary immunodeficiencies: dermatologic manifestations. J Invest Dermatol 1976 0.75
256 A diffuse cutaneous lymphoma secreting a potent immunosuppressant. Clin Immunol Immunopathol 1981 0.75
257 Evidence implicating calpain (Ca(2+)-dependent neutral protease) in the destructive thrombocytopenia of the Wiskott-Aldrich syndrome. Br J Haematol 1994 0.75
258 Immunoregulatory T-cell defects. Immunol Today 1983 0.75
259 GPL-115 deficiency: a new class of immunodeficiencies. Birth Defects Orig Artic Ser 1983 0.75
260 Lymphoid cell lines from patients with "non-secretory" agammaglobulinemia produce glycosylated heavy chains which are reduced in molecular weight. J Mol Cell Immunol 1984 0.75
261 Lymphocytotoxins and immunologic unresponsiveness. Birth Defects Orig Artic Ser 1975 0.75
262 John F. Enders (1897-1985). Nature 1985 0.75
263 Prognostic value of antibody-dependent assays for presensitization in bone marrow transplant recipients. Transplant Proc 1978 0.75
264 Induction of a specific antibody response in human B cells: role of a human antigenic nonspecific T-cell soluble factor and requirement for antigen. Cell Immunol 1977 0.75
265 Primary immunodeficiency diseases. R I Med J 1972 0.75
266 Gastrointestinal complications of immunodeficiency syndromes. Ciba Found Symp 1978 0.75
267 Diagnosis and treatment of antibody deficiency syndromes. Postgrad Med 1968 0.75
268 A high-resolution map of genes, microsatellite markers, and new dinucleotide repeats from UBE1 to the GATA locus in the region Xp11.23. Genomics 1995 0.75
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