Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Determinations of IgG subclasses were made by electroimmunoassay and crossed immunoelectrophoresis, and Gm markers were typed in sera from seventeen patients with well-defined immunodeficiency diseases. Certain IgG subclass and Gm patterns were recognized in various diseases: IgG2 deficiency and homozygosity of Gm (4,5) in the cartilage-hair-hypoplasia syndrome, in the ataxia telangiectasia syndrome and in selective IgG subclass deficiency; and IgG3 deficiency and homozygosity of Gm(1,-5) in the Wiskott-Aldrich syndrome. The findings suggest a common structural or regulator gene defect in some immunodeficiency diseases. In IgA deficiencies, the levels of IgG1 were raised. In patients with IgG subclass deficiencies there was sometimes a compensatory increase of the remaining IgG subclasses, with a preponderance of IgG1 and IgG3. The increased Ig1 showed restricted heterogeneity with only an increase of the electrophoretically cathodal part. This part contained both kappa and lambda chaings. IgG subclass deficiency indicates treatment with gammaglobulin even if the serum levels of IgG are normal or increased.
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PMID:Quantitative and qualitative investigations of serum IgG subclasses in immunodeficiency diseases. 46 57

IgG subclass levels were measured before and after administration of intravenous immunoglobulins (IVIGs) in patients with common variable immunodeficiency (CVI). Six patients were treated with IVIG at the dose of 100-150mg/kg to maintain a trough level of 200mg/dl every 4 or 5 weeks, except for patient 5 who was given IVIG only 3 or 4 times per year. Three kinds of IVIGs, polyethylene-glycol (PEG)-treated IVIG, alkylated IVIG and sulfonated IVIG were used for replacement therapy. Although serum IgGl levels were low before administration of IVIGs, they increased to the normal levels after each administration of IVIGs in four patients. IgG2 preserved normal levels before and after administration of IVIGs in all patients. IgG3 was present at low normal concentration in patient 1, low concentration in patients 2, 3 and 6, and undetectable in patients 4 and 5 before infusion. Although increases in IgG3 levels were shown after infusion of PEG-treated IVIG, there were no increases after infusion of sulfonated or alkylated IVIG. However, there have been several reports that IgG3 is detected in sulfonated or alkylated IVIG preparations by another method. IgG4 levels were somewhat low before administration, but four patients achieved normal serum levels with treatment. In light of the above results of replacement therapy with IVIGs, we should consider the IgG subclass levels for patients such as CVI or selective IgG subclass deficiency.
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PMID:Serum IgG subclass concentrations before and after administration of intravenous immunoglobulin in common variable immunodeficiency. 134 46

Passive immunity is conferred to the fetus by maternal antibodies, the majority of which are transported across the placenta during the third trimester of pregnancy. To determine the placental transport of anti-HIV-1 antibodies, serum from 5 women infected with human immunodeficiency virus (HIV) and their abortuses were examined for anti-HIV-1 antibodies. The gestational age of the abortuses ranged from 18 to 24 weeks and following polymerase chain reaction amplification, HIV-1 gag DNA was detected in tissue from 2 of the abortuses. The concentration of total IgG antibodies present in cord blood ranged from 2.9% to 12.5% of maternal levels. Antibodies directed against the envelope proteins, gp160 and gp120, the reverse transcriptase protein, p66, and the capsular protein, p24, were present in fetal and maternal serum. Although IgG1 was the predominant subclass antibody generated in response to HIV-1 proteins, IgG2, IgG3, and IgG4 directed against HIV-1 proteins were also detected. There were large differences in the antigens recognized by the antibodies produced in the mothers, and the IgG subclasses of the antibodies produced. HIV-1 proteins recognized by antibodies present in cord blood were similar to those recognized by paired maternal serum and IgG1, IgG2, IgG3 recognizing HIV-1 proteins were detected in fetal serum. However, there was a dichotomy in placental transport of IgG subclass antibodies to HIV-1 proteins. The role of these antibodies in prevention of vertical transmission of HIV-1 has yet to be determined.
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PMID:Characterization of IgG and IgG subclass antibodies present in paired maternal and fetal serum which are directed against HIV-1 proteins. 174 77

Protocols were evaluated in an attempt to produce human monoclonal antibodies (HumAb) specific for the human immunodeficiency virus type 1 (HIV-1). The first series of experiments involved in vitro immunization of normal human peripheral blood lymphocytes (PBL) with peptides C57 (HIV-1 strain IIIB clone BH10 gp 120 amino acids 324-338: GNMRQAHCNISRAKW) followed by either fusion to mouse/human heterohybrids or transformation with Epstein Barr virus (EBV). Using the hybridoma technology, three IgM class (lambda light chain) HumAb were obtained. In a parallel study, PBL from two HIV-1-infected patients were immortalized after in vitro stimulation with fragments of the HIV-1 envelope glycoprotein (recombinant gp120, the PB1 fragment of gp120, amino acids 295-473, or the penv9 fragment of gp160, amino acids 474-757). Five IgG class HumAb (three IgG2, lambda; one IgG1, K; one IgG3, lambda) reactive with the antigens used in the in vitro stimulations were obtained.
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PMID:Production of human monoclonal antibodies against HIV-1 peptides. 179 70

We found that a common amino acid sequence motif exists between the V3-loop region of the human immunodeficiency virus type I envelope protein HIV gp120 and the human immunoglobulin heavy chain variable regions of subclass III (Ig VH-III). In the Ig VH-III sequences, the common motif overlaps with framework-1, complementarity-determining-region-1 and framework-2. In the homologous regions, the two groups of sequences also have a similar distribution of residue variability. On the DNA sequence level, the homology includes the conserved rearrangement signals of the VH-III genes, which lends support to the speculation that the V3 region of gp120 also may be involved in rearrangement processes.
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PMID:Protein and DNA-sequence homologies between the V3-loop of human immunodeficiency virus type I envelope protein gp120 and immunoglobulin variable regions. 189 86

To analyze differential antibody responsiveness of potential pathogenetic significance, sera from 66 patients with human immunodeficiency virus-1 (HIV-1) infections at various Walter Reed (WR) stages of the disease were analyzed to determine the subclass distribution of HIV antibodies. Although all IgG subclasses were involved in the HIV antibody response, the frequency was highest for IgG1 and the lowest for IgG4. When IgG subclass responses to different HIV antigens were compared qualitatively, IgG1 was the major subclass reactive with env, pol, and gag antigens; IgG2 and IgG3 were almost equally represented in response to gag gene products; and IgG4 showed minimal reactivity to p24 antigen in all HIV-infected patients regardless of their clinical presentation. In contrast, significantly lower levels of IgG2 anti-gp41 were observed in patients at WR 5 and 6 (5%) when compared to those at stage WR 1 and 2 (88%). The IgG2 response to a recombinant gp 120/41 antigen, however, remained unchanged, suggesting that the lack of IgG2 response may be associated with lack of responsiveness to the carbohydrate epitope on gp41. Indeed, parallel measurements of IgG antibody responses to group A carbohydrate were also lower in patients at WR 5 and 6 stages, without affecting antibody responses to polyribosyl ribitol phosphate and phosphocholine. As antibody responses to group A carbohydrate with its N-acetyl D-glucosamine (GlcNAc) determinant were lower at the WR 5 and 6 stage of HIV disease, GlcNAc may be one of the antigenic determinants on gp41 that plays a critical role in some of the pathologic events of HIV infection.
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PMID:IgG subclass responses to human immunodeficiency virus-1 antigens: lack of IgG2 response to gp41 correlates with clinical manifestation of disease. 198 97

Infection with the human immunodeficiency virus (HIV) induces a polyclonal B-cell activation. Despite elevated serum immunoglobulin levels, a significant deterioration of the antigen specific humoral immune response exists in most cases. We studied the influence of HIV infection on the serum levels of IgG subclasses in children. We investigated 76 children (aged 15 months to 18 years) with HIV-1-infection. Most children (88%) showed elevated serum immunoglobulin levels. IgA (87%) and IgM (74%) were more often above normal levels for age than IgG (60%). IgG subclass serum levels were significantly altered. The increase in total IgG was mainly due to a marked augmentation of the IgG1 fraction. In most cases IgG3 was simultaneously elevated. Ten children (13%) had very low IgG4 levels (less than 0.03 g/l). Out of 61 patients older than 2 years 8 (13%) had a profound IgG2 deficiency with normal or elevated total IgG. Four of them also had low IgG4 levels (less than 0.03 g/l). A correlation between IgG2 deficiency and HIV infection according to the Centres for Disease Control classification for acquired immunodeficiency syndrome could not be demonstrated (three patients with symptomatic and five with asymptomatic infection).
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PMID:IgG2 deficiency in children with human immunodeficiency virus infection. 202 12

We purified IgG by an automated chromatographic procedure, using a recombinant DNA-produced Protein G ligand bound to silica as the solid phase. The IgG can be separated from human serum in approximately 20 min with little operator manipulation. Recovery studies indicate that unconcentrated eluates contain 77-100% of the applied IgG. Concentration to volumes approximating that of the applied serum resulted in recovery of 76-98% of the applied IgG. High-resolution protein electrophoresis of the eluates demonstrated retention of the broad gamma band, with virtual absence of other serum proteins. This finding was confirmed by immunoelectrophoresis, which demonstrated homogeneity of IgG in the eluates. IgG subclass distributions in four eluates, compared with the sera from which they were harvested, indicate comparable percentages of IgG1, IgG2, and IgG3 and some enhancement of the IgG4 fraction [serum IgG4: 2.9 +/- 0.4% (SEM); eluate IgG4: 13.8 +/- 1.7%, P = 0.007]. The reactivity of eluates from two sera containing various antibodies to human immunodeficiency virus type 1 was virtually identical to that of the sera from which they were prepared. We conclude that this chromatographic application is an effective purification method for serum IgG and antibodies of the IgG class. The method may be applicable in the specialty clinical laboratory, particularly in those interested in protein and other immunological abnormalities.
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PMID:Ligand affinity chromatographic separation of serum IgG on recombinant protein G-silica. 204 54

We studied serum concentrations of IgG subclasses in 47 human immunodeficiency virus 1-infected (17 asymptomatic and 30 symptomatic) children. Thirty-nine of 47 (83%) had an abnormality of at least 1 subclass. Sixteen had only elevated IgG1, 6 had only elevated IgG3 and 12 had elevated IgG1 and IgG3 concentrations. IgG2, IgG4 and combined IgG2-IgG4 deficiency was found in 3, 4 and 4 patients, respectively. IgG2 concentrations did not differ between patients with (n = 23) or without (n = 24) bacterial infections. Additionally the number of bacterial infections was similar between the patients with normal or low IgG2 and/or low IgG4. These data indicate that IgG subclass abnormalities are found in most children with human immunodeficiency virus 1 infection, but quantitative deficiencies of specific subclasses do not appear to explain the high frequency of bacterial infections occurring in these patients.
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PMID:Serum immunoglobulin G subclasses in children infected with human immunodeficiency virus type 1. 206 5

An ELISA procedure to determine the distribution of human IgG subclass antibodies directed against the gram-negative bacterium Branhamella catarrhalis has been developed using commercially available monoclonal anti-IgG subclass antibodies. Using whole bacteria as coating antigen the specificity of the assay was determined and showed minimal cross-reactivity with a range of other bacteria. Estimations of IgG1, IgG2, IgG3, IgG4 and total IgG antibodies directed against this antigen were performed. All normal adult sera tested had measurable antibody levels of specific IgG1, IgG2, IgG3 and total IgG. Specific IgG4 was undetectable in the majority of adult sera. These assays will be of value for investigation of both children and adults with suspected immunodeficiency and recurrent upper respiratory tract infection.
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PMID:An enzyme-linked immunosorbent assay for the determination of human IgG subclass antibodies directed against Branhamella catarrhalis. 210 16


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