Partial expression of Papillon-Lefèvre Syndrome.

Papillon- Lefèvre Syndrome (PLS) is a rare autosomal recessive trait, which is transmitted with an estimated frequency of one to four per million individuals. It is characterized by palmar plantar keratosis and severe early-onset periodontitis affecting both deciduous and permanent dentition. In this report, we present clinical, microbiological and leukocyte function test findings of a thirty-five year-old patient with symptoms typical of Papillon-Lefèvre Syndrome except for premature loss of deciduous and permanent dentition. The patient exhibited palmar plantar keratosis and an isolated, moderately deep periodontal pocket in the third quadrant. No anaerobic bacteria were isolated from the plaque culture. The neutrophil function test revealed defective chemotaxis and phagocytosis while intracellular killing and respiratory burst were normal.

Expression of macrophage inflammatory protein 1 alpha in the endothelial cells exposed to diamide / 华中科技大学学报（医学）（英德文版）

In order to study whether the endothelial cells (ECs) with lipid peroxidation induced by diamide can express and secrete macrophage inflammatory protein 1 alpha (MIP-1 alpha), the expression of MIP-1 alpha protein in the cells was detected by cell enzyme-linked immunosorbent assay (ELISA) and that of MIP-1 alpha mRNA was determined by cell in situ hybridization and nuclease S1 protection assay after the ECs were exposed to different concentrations of diamide for 4 h. The chemotactic activity of MIP-1 alpha was tested by micropore filter method using modified Boyden chambers. Cell ELISA showed that the expression of MIP-1 alpha protein in endothelial cells exposed to 1 mumol/L, 5 mumol/L and 10 mumol/L diamide was 1.9-fold, 2.3-fold and 1.7-fold respectively as much as that in the control cells, which was statistically significant by analysis of variance. In situ hybridization revealed that the mRNA expression of ECs treated with 1 mumol/L, 5 mumol/L and 10 mumol/L diamide was 1.3-fold, 3.0-fold and 1.7-fold as much as that in the control group, which had statistical significance (F = 188.93, P < 0.01). The mRNA expression in 5 mumol/L dimide treated ECs, measured by nuclease S1 protection assay, was 3.4-fold as much as that in the control group (t = 8.70, P < 0.05). Chemotactic response(99.50 +/- 4.31 microns) to the culture medium conditioned by 5 mumol/L diamide treated ECs, which was stronger than that(66.47 +/- 3.25 microns) conditioned by the ECs (F = 404.31, P < 0.05), was significantly decreased (F = 192.25, P < 0.05) after adding MIP-1 alpha antibody. It suggests that diamide, a lipid peroxidation inducer, could stimulate ECs to produce high level of MIP-1 alpha, and might play an important role in atherogenesis by promoting the migration of peripheral blood monocytes into arterial intima.

Lessons from tumor and immunocompetent cells. The quantitative engagement of ligand-receptor interactions modulates stop-and-go behavior as well as proliferation

The four main cell functions, proliferation, apoptosis, differentiation and migration, are tightly regulated by external signals that initiate intracellular signal transduction pathways and determine the cellular behaviour. The concentration and composition of such external signals are at least important for the decision of cells as to which function has to be executed. Interleukin-8 is a well known inducing signal for neutrophil granulocyte migration, while the epidermal growth factor is an inducing signal for breast carcinoma cell migration. Depending on the concentrations of interleukin-8, the neutrophil granulocytes are capable of migration. However, at high concentration of interleukin-8 the migratory activity of each single cell is reduced, indicating that high concentrations of the chemokine inhibit migration and promote the performance of other cell functions. Concerning breast carcinoma cells, the epidermal growth factor is not only an inducer of migration but also an inhibitor of proliferation. These two examples provide evidence for a dose dependent action of external signals for several cell functions in parallel. This versatility of the effects of one ligand might be based on several intracellular signal transduction pathways that are turned on. For the dose-dependent differences of the effect of interleukin-8 we propose a two wheel model of an inositolphosphate-mediated, ATP-independent release of calcium from intracellular stores and a cyclic AMP-mediated, ATP-dependent uptake of calcium into the endoplasmatic reticulum.

Quimiotaxis del neutrófilo en pacientes con periodontitis de aparición temprana / Neutrophil chemotaxis in patients with early-onset periodontitis

Se ha observado que el polimorfonuclear neutrófilo (PMN) es la célula de defensa por excelencia de las estructuras periodontales, al jugar un papel fundamental como primera línea de defensa del organismo ante el ataque bacteriano. Esta investigación se realizó para verificar si hay alteración en la función quimiotáctica de los neutrófilos en pacientes con periodontitis de aparición temprana (PAT). Se estudiaron 10 pacientes con PAT con buen estado de salud general, que no hubieran recibido terapia antibiótica en los últimos 6 meses y 10 sanos periodontal y sistémicamente, a los cuales se les tomó una muestra sanguínea de 10 cc para realizar la extracción de los PMN neutrófilos y posteriormente, la quimiotaxis bajo agarosa. El análisis estadístico realizado fue la prueba t para los datos paramétricos (edad, viabilidad, pureza y recuento) y la prueba U de Mann-Whitney para los no paramétricos (diferencial e índice quimiotáctico). Los pacientes con PAT no mostraron diferencias significativas en los valores de quimiotaxis al compararlos con los resultados de los sanos periodontalmente (p mayor 0.05). La edad promedio de los pacientes en ambos grupos fue de 23 años. Tanto la viabilidad como la pureza mínima requeridas para realizar la pureba de quimiotaxis fueron del 90 por ciento. De los resultados del presente estudio se concluye que no existe ninguna alteración en la función quimiotáctica del neutrófilo de sangre periférica de los pacientes con periodontitis de aparición temprana, sugiriéndose que, de existir alteración inmunológica en dichos pacientes, podría estar presente a otro nivel

Polymorphonuclear leukocyte functions enhanced by chemotaxis

Human polymorphonuclear leukocytes (PMN) migrate into tissues in response to chemoattractants, yet it is not known whether this process alters the functional capabilities of the PMN. Using recombinant human interleukin-8 (rHIL-8, 100 ng/ml) as a stimulus, we compared a population of PMN that migrated through a polyvinylpyrrolidone-coated polycarbonate filter containing 8.0 microns diameter pores with PMN stimulated in suspension. PMN were analyzed by flow cytometry according to functional and phenotypic criteria. CD11b/CD16 expression was unaltered by chemotaxis. In contrast, chemotaxis enhanced phagocytosis of E. coli, independent of opsonization with IgG. Similarly, chemotaxis increased baseline hydrogen peroxide production. We conclude that the chemotactic motion of PMN "primes" the cell for increased oxidative burst activity and augments the ability of PMN to ingest bacteria. This increased functional capability is distinct from rHIL-8 stimulation and appears to be independent of complement-and Fc-receptor expression.

Resposta fagocitária e atividade quimiotática de leucócitos mononucleares em crianças eutróficas / Phagocytic response and chemotaxic activity of mononuclear leukocytes in eutrophic children

A funçäo fagocitária e a atividade quimiotática de leucócitos mononucleares foram avaliadas em cinqüenta crianças eutróficas na faixa etária de dois a dez anos, com o objetivo de estabelecer curvas padröes

Quimiotaxis bajo agarosa: método para medir la quimiotaxis de los polimorfonucleares neutrofilos / Chemotaxis under agarose: method for measuring chemotaxis of polymorphonuclear neutrophils

Conocedores de la importancia de la quimiotaxis de los polimorfonucleares neutrófilos en el proceso de la fagocitosis y del número creciente de informes en la literatura sobre pacientes que presentan diversos cuadros clínicos relacionados con una deficiencia quimiotáctica, realizamos el presente estudio con el objeto de estandarizar una modificación de la técnica de quimiotaxis bajo agarosa descrita por Nelson y colaboradores