RESUMEN
28 human and 60 experimentally stimulated rabbit lymph nodes were studied by means of light microscopy and immunofluorescence. 21 of the 28 human lymph nodes showed well-developed germinal centers. IgM, IgG, and the beta(1C) component of complement were found in the same distribution within germinal centers when examined in serial cryostat sections. 36 rabbits were stimulated with Brucella antigen, and 24 rabbits with BSA. A strikingly consistent correlation between distribution and appearance of specific staining for rabbit beta(1C), IgM, and IgG was observed; when lymph nodes were stimulated with BSA, antigen and specific antibody were present. Treatment of unfixed sections with citrate-buffered saline at low pH resulted in complete elution of immunoglobulins, beta(1C), and BSA from rabbit germinal centers, and in marked diminution of IgG and IgM in human germinal centers, while at the same time plasma cells remained strongly fluorescent. Specific selective fixation of heterologous (human) complement in rabbit germinal centers positive for beta(1C), IgG, IgM, and BSA was also obtained. These data present strong evidence for the existence within germinal centers of antigen-antibody complexes which fix at least the beta(1C) component of complement in vivo. The possibility of complete elution of immunoglobulins from rabbit germinal centers can be taken as evidence that, at least for 20 days after primary and secondary stimulation, a major component of the immunoglobulins present in germinal centers is not produced locally but accumulates at the surface of cells.
Asunto(s)
Anticuerpos/análisis , Antígenos/análisis , Proteínas del Sistema Complemento/análisis , Inmunoglobulina G/análisis , Inmunoglobulina M/análisis , Ganglios Linfáticos/inmunología , Animales , Brucella , Eosinófilos/análisis , Técnica del Anticuerpo Fluorescente , Humanos , Ganglios Linfáticos/análisis , Linfocitos/análisis , Macrófagos/análisis , Métodos , Células Plasmáticas/análisis , Conejos , Albúmina Sérica BovinaRESUMEN
Lymphocytes in the bursa of chickens have been found to produce hemolytic antibodies to sheep erythrocytes that are introduced into the cloaca. These lymphocytes also react with Escherichia coli and form bacterial adherent colonies, but not with gamma streptococci to which they have not been previously exposed. Thymic and splenic lymphocytes do not bind either organism.
Asunto(s)
Formación de Anticuerpos , Células Productoras de Anticuerpos , Bolsa de Fabricio/inmunología , Linfocitos/inmunología , Animales , Reacciones Antígeno-Anticuerpo , Antígenos Bacterianos , Pollos , Eritrocitos/inmunología , Escherichia coli/inmunología , Técnica de Placa Hemolítica , Inmunización , Streptococcus/inmunologíaRESUMEN
In most instances, marked deficiency of the purine catabolic enzyme adenosine deaminase results in lymphopenia and severe combined immunodeficiency disease. Over a 2-yr period, we studied a white male child with markedly deficient erythrocyte and lymphocyte adenosine deaminase activity and normal immune function. We have documented that (a) adenosine deaminase activity and immunoreactive protein are undetectable in erythrocytes, 0.9% of normal in lymphocytes, 4% in cultured lymphoblasts, and 14% in skin fibroblasts; (b) plasma adenosine and deoxyadenosine levels are undetectable and deoxy ATP levels are only slightly elevated in lymphocytes and in erythrocytes; (c) no defect in deoxyadenosine metabolism is present in the proband's cultured lymphoblasts; (d) lymphoblast adenosine deaminase has normal enzyme kinetics, absolute specific activity, S20,w, pH optimum, and heat stability; and (e) the proband's adenosine deaminase exhibits a normal apparent subunit molecular weight but an abnormal isoelectric pH. In contrast to the three other adenosine deaminase-deficient healthy subjects who have been described, the proband is unique in demonstrating an acidic, heat-stable protein mutation of the enzyme that is associated with less than 1% lymphocyte adenosine deaminase activity. Residual adenosine deaminase activity in tissues other than lymphocytes may suffice to metabolize the otherwise lymphotoxic enzyme substrate(s) and account for the preservation of normal immune function.
Asunto(s)
Adenosina Desaminasa/deficiencia , Mutación , Nucleósido Desaminasas/deficiencia , Adenosina Desaminasa/sangre , Adenosina Desaminasa/inmunología , Formación de Anticuerpos , Preescolar , Reacciones Cruzadas , Desoxiadenosinas/sangre , Desoxiadenosinas/orina , Electroforesis en Gel de Poliacrilamida , Electroforesis en Gel de Almidón , Eritrocitos/enzimología , Humanos , Inmunidad Celular , Focalización Isoeléctrica , Activación de Linfocitos , Linfocitos/enzimología , MasculinoRESUMEN
Patients with Wiskott-Aldrich Syndrome have an increased incidence of serious infections, often with microorganisms that usually produce mild disease in immunologically normal subjects. Three patients with Wiskott-Aldrich syndrome complicated by progressive varicella are reported. There have been no previous reports of similar cases. Two of the patients were treated with adenine arabinoside and had rapid recovery.
Asunto(s)
Varicela/tratamiento farmacológico , Vidarabina/uso terapéutico , Síndrome de Wiskott-Aldrich/complicaciones , Adolescente , Anticuerpos Antivirales/análisis , Varicela/etiología , Varicela/inmunología , Varicela/prevención & control , Niño , Humanos , Sueros Inmunes/administración & dosificación , Inmunización Pasiva , Lactante , MasculinoAsunto(s)
Agammaglobulinemia/complicaciones , Histoplasmosis/inmunología , Enfermedades Pulmonares Fúngicas/inmunología , Histoplasmosis/diagnóstico por imagen , Histoplasmosis/patología , Humanos , Inmunidad Celular , Pulmón/patología , Enfermedades Pulmonares Fúngicas/diagnóstico por imagen , Enfermedades Pulmonares Fúngicas/patología , Masculino , Persona de Mediana Edad , RadiografíaAsunto(s)
Células de la Médula Ósea , Trasplante de Médula Ósea , Agammaglobulinemia/terapia , Anemia Aplásica/terapia , Suero Antilinfocítico , Femenino , Enfermedad Injerto contra Huésped/tratamiento farmacológico , Enfermedad Injerto contra Huésped/inmunología , Antígenos de Histocompatibilidad , Prueba de Histocompatibilidad , Enfermedad de Hodgkin/terapia , Humanos , Inmunidad Celular , Inmunoglobulinas/análisis , Inmunosupresores/uso terapéutico , Lactante , Leucemia Linfoide/terapia , Prueba de Cultivo Mixto de Linfocitos , Linfocitos/inmunología , Linfopenia/terapia , Masculino , Mitomicinas , Trasplante HomólogoAsunto(s)
Línea Celular , Reacción Injerto-Huésped , Linfocitos/inmunología , Animales , Femenino , Técnica del Anticuerpo Fluorescente , Caballos/inmunología , Humanos , Sueros Inmunes , Leucemia Mieloide Aguda/inmunología , Transfusión de Linfocitos , Linfoma/inmunología , Trasplante de Neoplasias , Ratas , Antitoxina Tetánica , Trasplante HeterólogoAsunto(s)
Células Productoras de Anticuerpos/inmunología , Células de la Médula Ósea , Médula Ósea/inmunología , Linfocitos T/inmunología , Animales , Trasplante de Médula Ósea , Radioisótopos de Carbono , Legrado , Reacción Injerto-Huésped , Haplorrinos , Humanos , Inhalación , Lectinas , Macaca , Mitógenos , Estimulación Química , Linfocitos T/análisis , Trasplante HomólogoAsunto(s)
Linfocitos/inmunología , Células Cultivadas , Diatrizoato , Humanos , Técnicas In Vitro , Fagocitosis , Polisacáridos , Estudios ProspectivosAsunto(s)
Lectinas/farmacología , Linfocitos/fisiología , Mitomicinas/farmacología , Mitosis/efectos de los fármacos , Timo/inmunología , Animales , Inmunoquímica , Técnicas In Vitro , Linfocitos/inmunología , Linfocitos/metabolismo , Ratas , Timectomía , Timidina/metabolismo , Timo/fisiología , TritioAsunto(s)
Trasplante de Médula Ósea , Reacción Injerto-Huésped , Autopsia , Biopsia , Médula Ósea/patología , Bronconeumonía/patología , Ciclofosfamida/efectos adversos , Femenino , Gastroenteritis/inducido químicamente , Gastroenteritis/patología , Células Madre Hematopoyéticas , Prueba de Histocompatibilidad , Enfermedad de Hodgkin/patología , Humanos , Hialina , Síndromes de Inmunodeficiencia/patología , Lactante , Ganglios Linfáticos/patología , Linfocitos , Masculino , Microscopía , Úlcera Péptica/inducido químicamente , Células Plasmáticas , Neumonía por Pneumocystis/patología , Piel/patología , Enfermedades de la Piel/patología , Bazo/patología , Estómago/patología , Timo/patología , Trasplante HomólogoAsunto(s)
Células de la Médula Ósea , Trasplante de Médula Ósea , Síndromes de Inmunodeficiencia/terapia , Terapia de Inmunosupresión , Adenosina , Aminohidrolasas , Anemia Aplásica/terapia , Suero Antilinfocítico/uso terapéutico , Niño , Ciclofosfamida/uso terapéutico , Reacción Injerto-Huésped , Prueba de Histocompatibilidad , Humanos , Leucemia/terapia , Masculino , Errores Innatos del Metabolismo , Mutación , Neoplasias/tratamiento farmacológico , Neoplasias/radioterapia , Timo/trasplanteAsunto(s)
Síndromes de Inmunodeficiencia/diagnóstico , Adulto , Linfocitos B/inmunología , Niño , Proteínas del Sistema Complemento/análisis , Humanos , Inmunoglobulinas/análisis , Síndromes de Inmunodeficiencia/genética , Síndromes de Inmunodeficiencia/inmunología , Técnicas Inmunológicas , Lactante , Nitroazul de Tetrazolio , Fagocitosis , Pruebas Cutáneas , Linfocitos T/inmunologíaRESUMEN
A number of infants with an autosomal recessive form of combined immunodeficiency disease also lack adenosine deaminase (adenosine aminohydrolase; EC 3.5.4.4) activity in their erythrocytes. Other tissues from these infants contain only a few percent of the adenosine-deaminating activity present in corresponding normal tissue. The residual adenosine-deaminating activity in extracts from the spleen of a combined immunodeficient, adenosine deaminase-deficient patient was compared with adenosine deaminase from normal spleen. Affinity and immunoadsorbant column chromatography revealed distinct differences between the adenosine-deaminating activity in the patient's spleen and adenosine deaminase from normal spleen. The point of maximum activity and general configuration of the pH optimum curves were also different. erythro-9-(2-Hydroxyl-3-nonyl)adenine, a potent inhibitor of adenosine deaminase from normal spleen, had relatively little effect on the activity from the patient's spleen. In contrast, adenine was a better inhibitor of the activity in the patient's spleen than it was of the enzyme from normal tissue. An adenosine-deaminating activity with the same characteristics and specific activity as that in the patient's spleen was also isolated from normal spleen. These results suggest that the adenosine-deaminating activity in the spleen of this patient is not due to a mutant form of adenosine deaminase.
Asunto(s)
Adenosina Desaminasa/metabolismo , Síndromes de Inmunodeficiencia/enzimología , Nucleósido Desaminasas/metabolismo , Bazo/enzimología , Adenina/farmacología , Adenosina/análogos & derivados , Adenosina/farmacología , Adenosina Desaminasa/sangre , Adenosina Desaminasa/deficiencia , Adenosina Desaminasa/inmunología , Reacciones Cruzadas , Concentración de Iones de Hidrógeno , CinéticaRESUMEN
Fifty-five children with CID and known ADA status were studies at a workshop held in Albany, New York. Erythrocyte ADA determinations were performed in 22 of the 55 patients, 13 of whom were ADA negative. The ADA defect appears to be transmitted as an autosomal recessive trait. Some patients with CID and ADA deficiency have characteristic radiologic abnormalities of the skeleton, which are not found in other illnesses. The thymus glands of all patients with CID and ADA deficiency who could be examined have evidence of thymic involution manifested by presence of Hassall's corpuscles and differentiated germinal epithelium; this is in contrast to "classic" thymus findings in CID with normal ADA. Adenosine deaminase probably plays an important, although as yet undefined, role in lymphocyte development and/or function. The deficiency of ADA in CID is the first enzyme defect observed in a deficiency disease of specific immunity.
Asunto(s)
Aminohidrolasas/deficiencia , Síndromes de Inmunodeficiencia/complicaciones , Adenosina , Aminohidrolasas/análisis , Aminohidrolasas/sangre , Linfocitos B/enzimología , Eritrocitos/enzimología , Femenino , Feto , Fibroblastos/enzimología , Genes Recesivos , Humanos , Húmero/diagnóstico por imagen , Íleon/diagnóstico por imagen , Síndromes de Inmunodeficiencia/enzimología , Síndromes de Inmunodeficiencia/patología , Trasplante de Hígado , Masculino , Miocardio/enzimología , New York , Pelvis/diagnóstico por imagen , Radiografía , Bazo/enzimología , Linfocitos T/enzimología , Timo/patología , Timo/trasplanteRESUMEN
Deficiency of red cell and lymphocyte adenosine deaminase (ADA) was found in children suffering from congenital combined immunologic deficiency. The parents had ADA levels intermediate between patients and controls. Complete lack of ADA activity was not found in normal subjects or in patients with a variety of other immunologic deficiency diseases.
Asunto(s)
Adenosina Desaminasa/deficiencia , Síndromes de Inmunodeficiencia/enzimología , Nucleósido Desaminasas/deficiencia , Adenosina Desaminasa/sangre , Niño , Cromosomas Humanos 16-18 , Eritrocitos/enzimología , Femenino , Genes , Humanos , Síndromes de Inmunodeficiencia/genética , Linfocitos/enzimología , Purinas/metabolismo , EspectrofotometríaRESUMEN
Sera from patients with chronic renal failure (CRF) contain a factor(s) which enhances the oxidative metabolism of polymorphonuclear leukocytes (PMN) as assessed by chemiluminescence (CL), superoxide anion generation, and hexose monophosphate shunt activity. PMN oxidative metabolic activity was higher in CRF sera than in sera from hospitalized patients with normal renal function or in sera from normal healthy subjects. The enhancement occurred regardless of whether PMN were unstimulated or were stimulated by a nonspecific soluble membrane stimulant (phorbol myristate acetate), or by opsonized Candida albicans. The enhanced CL was significantly reduced in their sera after normal renal function was restored with successful renal transplantation. This CL-enhancing factor was also detected in dialysate fluids from CRF patients and in urine from normal healthy subjects. When serum, urine, dialysate fluids of these CRF patients were fractionated by Sephadex G-25 column chromatography, the specific fraction responsible for enhanced CL was found in the molecular weight range less than 1,000 daltons, and is an ethanol extractable substance with natural fluorescence. Our findings suggest that the enhanced PMN stimulatory activity in CRF serum is specifically associated with renal dysfunction and can be useful, along with other conventional parameters, for monitoring the progression of CRF.