Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 149
Filtrar
Más filtros

Intervalo de año de publicación
1.
J Craniofac Surg ; 2024 Feb 16.
Artículo en Inglés | MEDLINE | ID: mdl-38363319

RESUMEN

Blepharoplasty is a commonly performed cosmetic surgery worldwide to address the excess skin and fat on the upper eyelids. The purpose of this paper is to present a surgical variation on blepharoplasty, achieving more pleasant outcomes. This paper describes the cauterization + plication technique, which involves using a cauterizing tool to remove excess skin and fat from the upper eyelid before using sutures to create a crease. It provides valuable insights into the advantages and limitations of this technique, drawing on relevant literature and clinical experience. Muscle cauterization and plication technique is an upgrade from the traditional technique, producing more esthetically pleasing results. This study highlights the importance of understanding the different techniques available for blepharoplasty to achieve the best possible outcomes for patients.

2.
Eur Cell Mater ; 41: 502-516, 2021 05 10.
Artículo en Inglés | MEDLINE | ID: mdl-33970477

RESUMEN

Genetic conditions, traumatic injuries, carious lesions and periodontal diseases are all responsible for dental pathologies. The current clinical approaches are based on the substitution of damaged dental tissues with inert materials, which, however, do not ensure full physiological recovery of the teeth. Different populations of dental mesenchymal stem cells have been isolated from dental tissues and several attempts have already been made at using these stem cells for the regeneration of human dental tissues. Despite encouraging progresses, dental regenerative therapies are very far from any clinical applications. This is tightly connected with the absence of proper platforms that would model and faithfully mimic human dental tissues in their complexity. Therefore, in the last decades, many efforts have been dedicated for the development of innovative systems capable of emulating human tooth physiology in vitro. This review focuses on the use of in vitro culture systems, such as bioreactors and "organ-on-a-chip" microfluidic devices, for the modelling of human dental tissues and their potential use for dental regeneration and drug testing.


Asunto(s)
Regeneración/fisiología , Diente/fisiología , Animales , Humanos , Células Madre Mesenquimatosas/fisiología , Ingeniería de Tejidos/métodos
3.
J Biol Regul Homeost Agents ; 33(6): 1715-1723, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-31797649

RESUMEN

Ascorbic acid (AS), also known as vitamin C or ascorbate, is an essential dietary nutrient which plays a vital role in biological processes through various different mechanisms, in particular for the biosynthesis of collagen. The aim of the study was to establish the possibility of enhancing the osteogenic differentiation potential by manipulating the cellular micro-environment, through AS supplementation in human gingival mesenchymal stem cells (hGMSCs) at different concentrations, such as 60 and 90 µg/mL, for three weeks. Human GMSCs are considered a stem cell population, easily obtainable and displaying a remarkable immunotherapeutic potential and regenerative repair expression. Osteogenic differentiation level induced from AS was assayed by histochemical characterization, using light microscopy through Alizarin red S staining. The transcript levels of Collagen 1A1 (COL1A1), runtrelated transcription factor 2 (RUNX2), bone morphogenetic protein 2/4 (BMP2/4), osteopontin (OPN) and osteonectin (SPARC) were determined by quantitative RT-PCR. Protein expression of COL1A1, RUNX2, BMP2/4, OPN, SPARC were studied through Western blotting and confocal laser scanning microscopy (CLSM). Our results demonstrate that AS supports osteogenic differentiation in stem cells from gingiva niche as shown by osteogenic marker upregulation and by de novo production of calcium phosphate deposits as revealed by Alizarin red S staining. In summary, the results of the current study provide evidence that hGMSCs undergo osteogenic differentiation with AS treatment, for that reason AS could be a promising candidate for the prevention and healing of bone-related diseases.


Asunto(s)
Ácido Ascórbico/farmacología , Diferenciación Celular , Células Madre Mesenquimatosas/citología , Osteogénesis , Células Cultivadas , Encía/citología , Humanos
4.
Int Endod J ; 48(9): 839-49, 2015 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-25231818

RESUMEN

AIM: To evaluate the effect of TEGDMA on human gingival fibroblasts (HGFs) in vitro co-cultured with Streptococcus mitis, focusing on the signalling pathways underlying cell tissue remodelling and inflammatory response processes. METHODOLOGY: ß1 integrin expression was evaluated by means of imaging flow cytometry. The Western blot technique was used to investigate the expression of protein kinase C (PKC), extracellular signal-regulated kinase (ERK), matrix metalloproteinase 9 (MMP9) and 3 (MMP3). RT-PCR was performed to quantify nuclear factor-kb subunits (Nf-kb1, ReLa), IkB kinase ß (IkBkB), cyclooxygenase II (COX-2) and tumour necrosis factor-α (TNF-α) mRNA levels. Statistical analysis was performed using the analysis of variance (anova). RESULTS: When HGFs are co-cultured with S. mitis, ß1 integrin intensity, phosphorylated PKC (p-PKC), activated ERK (p-ERK), IkBkB mRNA level and MMP9 expression increased (for all molecules P < 0.05 HGFs versus HGFs co-cultured with S. mitis). A higher level of MMP3 in HGFs treated with TEGDMA was recorded (P < 0.05 HGFs versus HGFs exposed to TEGDMA). COX-2 inflammatory factor mRNA level appeared higher in HGFs exposed to 1 mmol L(-1) TEGDMA (P < 0.01 HGFs versus HGFs exposed to TEGDMA), whereas TNF-α gene expression was higher in HGFs co-cultured with S. mitis (P < 0.05 HGFs versus HGFs co-cultured with S. mitis). CONCLUSIONS: ß1 integrin triggered the signalling pathway, transduced by p-PKCα and involving ERK 1 and 2 and MMPs. This pathway resulted in an unbalanced equilibrium in tissue remodelling process, along with inflammatory response when HGFs are exposed to bacteria or biomaterial alone. On the contrary, the TEGDMA/S. mitis combination restored the balance between extracellular matrix deposition and degradation and prevented an inflammatory response.


Asunto(s)
Fibroblastos/efectos de los fármacos , Encía/efectos de los fármacos , Polietilenglicoles/farmacología , Ácidos Polimetacrílicos/farmacología , Streptococcus mitis/efectos de los fármacos , Técnicas de Cocultivo , Fibroblastos/citología , Fibroblastos/enzimología , Encía/citología , Encía/enzimología , Humanos , Inflamación/metabolismo , Integrina beta1/metabolismo , Proteína Quinasa C-alfa/metabolismo , Transducción de Señal , Streptococcus mitis/fisiología , Factor de Necrosis Tumoral alfa/metabolismo
5.
Genome ; 56(3): 155-9, 2013 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-23659699

RESUMEN

The European hazelnut (Corylus avellana L.) is widespread in Europe, where it has been cultivated for centuries. Despite progress in genetics, most of the cytogenetic aspects of this species have been overlooked. The aim of this study was to fill in this gap and obtain basic information on the chromosome structure of this species. Karyomorphological analysis confirmed the chromosome number 2n = 22 and showed that, despite their apparent uniformity, the chromosomes could be separated into three groups of different size: large (L), medium (M), and small (S). As a first step towards the physical mapping of the hazelnut chromosomes, we applied FISH to localize the position of rRNA genes (rDNA). The sites of 45S and 5S rDNA enabled us to identify two chromosome pairs belonging, respectively, to the L and S groups. The self-GISH procedure revealed that repetitive DNA is concentrated in the pericentromeric regions of the chromosomes, as with other species with rather small genomes. The analysis of 5S rDNA repeats offered additional information on the hazelnut genome by obtaining the whole sequence of the transcribed region so far unpublished. The overall results constitute a substantial advance in hazelnut cytogenetics. Further investigation of other species of Corylus could be an effective approach to understanding the phylogenesis of the genus and resolving taxonomic problems.


Asunto(s)
Corylus/genética , Hibridación Fluorescente in Situ , Cariotipo , ARN Ribosómico 5S/genética , Secuencia de Bases , Mapeo Cromosómico , Cromosomas de las Plantas , Europa (Continente) , Datos de Secuencia Molecular , ARN Ribosómico 5S/química
6.
Acta Anaesthesiol Scand ; 57(7): 929-35, 2013 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-23701337

RESUMEN

BACKGROUND: Early recognition of hypovolaemia in trauma patients is very important. However, the most often used clinical signs, such as hypotension and tachycardia, lack specificity and sensitivity. METHODS: We propose a non-invasive index of hypovolaemia, the heart to arm time (iHAT), based on a modified pulse transit time indexed to heart rate. Pulse transit time is the sum of pre-ejection period and vascular transit time. Following pre-load reductions due to hypovolaemia, ventricular diastolic filling time increases causing an increase in pre-ejection-period, pulse transit time, and hence iHAT. One hundred and four consecutive patients with suspected major trauma were enrolled. The primary aim was to evaluate the use of the iHAT for detecting haemorrhage in major trauma. The secondary end point was to compare the specificity and sensitivity of iHAT compared to commonly used indexes. RESULTS: iHAT was calculated in 84 subjects, 11 of whom were haemorrhagic. iHAT discriminated haemorrhagic from non-haemorrhagic group (46.8% vs. 66.9%, P < 0.0001). The cut-off for iHAT with the best compromise between sensitivity (90.9%) and specificity (100%) was reached at the 58.78% level. Comparing haemorrhagic and non-haemorrhagic patients, the area under the ROC curve was 0.952 for iHAT, 0.835 for heart rate, and 0.911 for systolic blood pressure, showing no significant differences. CONCLUSIONS: iHAT is a non-invasive index that can identify haemorrhage in trauma patients with high sensitivity and specificity. These data should be considered as an exploration, but any conclusion should be validated in a new set of consecutive patients.


Asunto(s)
Brazo/irrigación sanguínea , Técnicas de Diagnóstico Cardiovascular , Servicios Médicos de Urgencia/métodos , Frecuencia Cardíaca , Hemorragia/diagnóstico , Pulso Arterial , Heridas y Lesiones/complicaciones , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Algoritmos , Área Bajo la Curva , Femenino , Hemorragia/etiología , Hemorragia/terapia , Humanos , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Curva ROC , Sensibilidad y Especificidad , Choque/diagnóstico , Choque/etiología , Choque/prevención & control , Factores de Tiempo , Procedimientos Innecesarios , Heridas y Lesiones/fisiopatología , Adulto Joven
7.
Nat Genet ; 20(1): 37-42, 1998 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-9731527

RESUMEN

The limb-girdle muscular dystrophies are a genetically heterogeneous group of inherited progressive muscle disorders that affect mainly the proximal musculature, with evidence for at least three autosomal dominant and eight autosomal recessive loci. The latter mostly involve mutations in genes encoding components of the dystrophin-associated complex; another form is caused by mutations in the gene for the muscle-specific protease calpain 3. Using a positional cloning approach, we have identified the gene for a form of limb-girdle muscular dystrophy that we previously mapped to chromosome 2p13 (LGMD2B). This gene shows no homology to any known mammalian gene, but its predicted product is related to the C. elegans spermatogenesis factor fer-1. We have identified two homozygous frameshift mutations in this gene, resulting in muscular dystrophy of either proximal or distal onset in nine families. The proposed name 'dysferlin' combines the role of the gene in producing muscular dystrophy with its C. elegans homology.


Asunto(s)
Proteínas de Caenorhabditis elegans , Caenorhabditis elegans/genética , Proteínas del Helminto/genética , Proteínas de la Membrana , Proteínas Musculares/genética , Distrofias Musculares/genética , Mutación , Adolescente , Adulto , Secuencia de Aminoácidos , Animales , Niño , Mapeo Cromosómico , Cromosomas Artificiales de Levadura , Cromosomas Humanos Par 2 , Disferlina , Femenino , Humanos , Masculino , Datos de Secuencia Molecular , Proteínas Musculares/química , Proteínas Musculares/metabolismo , Linaje , Homología de Secuencia de Aminoácido , Distribución Tisular
8.
Plant Reprod ; 2023 Dec 06.
Artículo en Inglés | MEDLINE | ID: mdl-38055074

RESUMEN

Epigenetics studies changes in gene activity without changes in the DNA sequence. Methylation is an epigenetic mechanism important in many pathways, such as biotic and abiotic stresses, cell division, and reproduction. Eragrostis curvula is a grass species reproducing by apomixis, a clonal reproduction by seeds. This work employed the MCSeEd technique to identify deferentially methylated positions, regions, and genes in the CG, CHG, and CHH contexts in E. curvula genotypes with similar genomic backgrounds but with different reproductive modes and ploidy levels. In this way, we focused the analysis on the cvs. Tanganyika INTA (4x, apomictic), Victoria (2x, sexual), and Bahiense (4x, apomictic). Victoria was obtained from the diploidization of Tanganyika INTA, while Bahiense was produced from the tetraploidization of Victoria. This study showed that polyploid/apomictic genotypes had more differentially methylated positions and regions than the diploid sexual ones. Interestingly, it was possible to observe fewer differentially methylated positions and regions in CG than in the other contexts, meaning CG methylation is conserved across the genotypes regardless of the ploidy level and reproductive mode. In the comparisons between sexual and apomictic genotypes, we identified differentially methylated genes involved in the reproductive pathways, specifically in meiosis, cell division, and fertilization. Another interesting observation was that several differentially methylated genes between the diploid and the original tetraploid genotype recovered their methylation status after tetraploidization, suggesting that methylation is an important mechanism involved in reproduction and ploidy changes.

9.
J Exp Med ; 183(1): 203-13, 1996 Jan 01.
Artículo en Inglés | MEDLINE | ID: mdl-8551224

RESUMEN

The mechanisms that induce T cell tolerance to circulating self-proteins are still controversial, and both the deletion and selection of autoreactive T cells have been observed in the thymus of transgenic mouse models. To address the question of the induction of tolerance to circulating self-constituents, a T cell receptor-transgenic mouse specific for the serum protein immunoglobulin (Ig) gamma and (IgG2ab) was generated. The choice of an allotype-specific T cell also allowed the generation of transgenic control mice not expressing the self-antigen. It was found that the transgenic T cells were not deleted in the thymus, did not become tolerant in the periphery, and regulated the function of gamma 2ab-positive B cells as shown by the lack of IgG2ab protein in the serum of the transgenic mice. In spite of this activity in vivo, the transgenic T cells did not proliferate in vitro in response to the allotype-specific peptide. Interestingly, antigen-specific T cell proliferation could be restored if the transgenic mice were previously challenged to induce IgG2ab responses. After this challenge, IgG2ab protein in the serum of the transgenic mice could be partially restored, although still remaining much lower than in control mice. In addition, there was a dramatic increase in serum IgE levels, suggesting that newly generated gamma 2ab-secreting B cells can be induced to switch to IgE in the presence of allotype-specific T cells. These results indicate that Ig-specific T cells may represent a late-acting form of T cell help for the regulation of the IgG2a-to-IgE class switch.


Asunto(s)
Tolerancia Inmunológica , Receptores de Antígenos de Linfocitos T/inmunología , Receptores de IgG/inmunología , Subgrupos de Linfocitos T/inmunología , Timo/inmunología , Animales , Secuencia de Bases , Antígenos CD4/inmunología , Antígenos CD8/inmunología , Supresión Clonal/inmunología , Citocinas/análisis , Citometría de Flujo , Inmunización , Isotipos de Inmunoglobulinas/análisis , Isotipos de Inmunoglobulinas/inmunología , Activación de Linfocitos , Ratones , Ratones Endogámicos BALB C , Ratones Endogámicos DBA , Ratones Transgénicos , Modelos Inmunológicos , Datos de Secuencia Molecular , Péptidos/inmunología , Receptores de Antígenos de Linfocitos T/genética , Receptores de IgG/genética , Salmonella typhimurium/inmunología , Autotolerancia/inmunología , Timectomía , Timo/citología , Timo/cirugía
10.
Allergy ; 65(10): 1313-21, 2010 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-20374228

RESUMEN

BACKGROUND: Pollution is considered as one main cause for the increase of allergic diseases. Air pollutants may cause and worsen airway diseases and are probably able to make pollen allergens more aggressive. Previous studies looked at traffic-related air pollution, but no data about the effects of polluted soils on pollen allergens are available. We aimed to assess the effects of plant exposure to cadmium-contaminated soil on allergenicity of the annual blue grass, Poa annua L, pollen. METHODS: Poa plants were grown in soil contaminated or not contaminated (control) with cadmium. At flowering, mature pollen was analyzed by microscopy, to calculate the percentage of pollen grains releasing cytoplasmic granules, and by proteomic techniques to analyze allergen proteins. Allergens were identified by sera from grass pollen-allergic patients and by mass spectrometry. RESULTS: Pollen from Cd-exposed plants released a higher amount of allergenic proteins than control plants. Moreover, Cd-exposed pollen released allergens-containing cytoplasmic grains much more promptly than control pollen. Group 1 and 5 allergens, the major grass pollen allergens, were detected both in control and Cd-exposed extracts. These were the only allergens reacting with patient's sera in control pollen, whereas additional proteins strengthening the signal in the gel region reacting with patient's sera were present in Cd-exposed pollen. These included a pectinesterase, a lipase, a nuclease, and a secretory peroxydase. Moreover, a PR3 class I chitinase-like protein was also immunodetected in exposed plants. CONCLUSION: Pollen content of plants grown in Cd-contaminated soils is more easily released in the environment and also shows an increased propensity to bind specific IgE.


Asunto(s)
Cadmio/farmacología , Exposición a Riesgos Ambientales/efectos adversos , Hipersensibilidad/etiología , Poa/inmunología , Polen/inmunología , Contaminantes del Suelo/farmacología , Adulto , Alérgenos/análisis , Alérgenos/sangre , Alérgenos/efectos de los fármacos , Cadmio/metabolismo , Humanos , Inmunoglobulina E/inmunología , Espectrometría de Masas , Poa/efectos de los fármacos , Poa/metabolismo , Polen/efectos adversos , Contaminantes del Suelo/metabolismo
11.
J Clin Invest ; 99(7): 1691-8, 1997 Apr 01.
Artículo en Inglés | MEDLINE | ID: mdl-9120013

RESUMEN

Myotonic dystrophy is a dominantly inherited clinically variable multisystemic disorder, and has been found to be caused by heterozygosity for a trinucleotide repeat expansion mutation in the 3' untranslated region of a protein kinase gene (DM kinase). The mechanisms by which the expanded repeat in DNA results in a dominant biochemical defect and the varied clinical phenotype, is not known. We have recently proposed a model where disease pathogenesis may occur at the RNA level in myotonic dystrophy: the mutant DM kinase RNA with the expansion mutation may disrupt cellular RNA metabolism in some general manner, as evidenced by defects in RNA processing of the normal DM kinase gene in heterozygous patients (dominant negative RNA mutation). Here we further test this hypothesis by measuring RNA metabolism of other genes in patient muscle biopsies (nine adult onset myotonic dystrophy patients, two congenital muscular dystrophy patients, four normal controls, and four myopathic controls). We focused on the insulin receptor gene because of the documented insulin resistance of DM patients. We show that there is a significant decrease in insulin receptor RNA in both total RNA and RNA polyA+ pools relative to normal and myopathic control muscles (P < 0.002), measured relative to both dystrophin RNA and muscle sodium channel RNA. We also show reductions in insulin receptor protein. Our results reinforce the concept of a generalized RNA metabolism defect in myotonic dystrophy, and offer a possible molecular mechanism for the increased insulin resistance observed in many myotonic dystrophy patients.


Asunto(s)
Resistencia a la Insulina , Músculos/metabolismo , Distrofia Miotónica/metabolismo , ARN/metabolismo , Receptor de Insulina/genética , Adolescente , Adulto , Niño , Humanos , Lactante , Persona de Mediana Edad , Proteína Quinasa de Distrofia Miotónica , Proteínas Serina-Treonina Quinasas/genética , ARN/análisis , Receptor de Insulina/análisis
12.
Transplant Proc ; 38(4): 1074-5, 2006 May.
Artículo en Inglés | MEDLINE | ID: mdl-16757268

RESUMEN

Small-for-size syndrome occurs in the presence of a reduced mass of liver that is insufficient to maintain normal liver function. It has been speculated that this dysfunction is principally associated with graft exposure to excessive portal perfusion. The aim of these cases was to evaluate the efficacy of octreotide, a splanchnic vasoconstrictor, and esmolol, a selective beta-blocker, to modify the portal perfusion in the postoperative phase after left living related liver transplantation (LRLT). Four patients who underwent left LRLT with graft-to-recipient weight ratios of 0.60 +/- 0.24 were studied with a catheter placed in a jejunal vein. We observed high basal values of hepatic venous pressure gradient (HVPG) and portal vein flow (PVF). Octreotide infusion decreased HVPG, an effect that was more pronounced when it was combined with esmolol. The administration of both drugs was also associated with an improvement in portal vein oxygen saturation. Despite variation in PVF, the plasma disappearance rate of indocyanin green did not change during the infusion of the two drugs. In conclusion, octreotide and esmolol infusion allowed a manipulation of portal vein pressure that should be measured in left LRLT using a small-for-size graft.


Asunto(s)
Presión Sanguínea/fisiología , Hepatectomía/métodos , Trasplante de Hígado/fisiología , Donadores Vivos , Vena Porta/fisiología , Obtención de Tejidos y Órganos/métodos , Familia , Femenino , Humanos , Masculino , Persona de Mediana Edad , Octreótido/farmacología , Selección de Paciente , Vena Porta/efectos de los fármacos , Flujo Sanguíneo Regional/efectos de los fármacos
13.
Oncogene ; 9(5): 1473-7, 1994 May.
Artículo en Inglés | MEDLINE | ID: mdl-8152810

RESUMEN

The VN-11 recombinant retroviruses, originally generated by co-transfection of the avian MH2 and AKRv viral genomes, were molecularly cloned from an infected mouse cell line named N11. The analysis of the proviral genome sequence from one of these recombinants showed a possible envAKR-mycMH2 fusion. Point mutations were also found in this envAKR-mycMH2 gene. The cloned viral genome was co-transfected with the neo gene into the psi 2 packaging cell line. Selected clones were shown to transcribe the viral genome and supernatants from these cultures, containing C-type particles, were used to infect primary cultures from mouse lymphoid tissues and brain. Proliferating macrophages and microglial cell clones were obtained, indicating that various types of cells of the mouse monocytic-macrophage lineage can be immortalized in spite of the absence of selection or special growth conditions.


Asunto(s)
Clonación Molecular , Genes env , Genes myc , Macrófagos/citología , Monocitos/citología , Retroviridae/genética , Animales , Secuencia de Bases , División Celular , Línea Celular , Genoma Viral , Ratones , Datos de Secuencia Molecular , Mutación/genética
14.
Int J Radiat Biol ; 81(4): 319-26, 2005 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-16019941

RESUMEN

The aim was to obtain information on the one-electron reduction of the antimalarial natural drug artemisinin (ART). The pulse radiolysis of ART in H(2)O/ethanol (EtOH) (1:1 v/v) solution was studied in the absence and presence of the so-called redox indicators N,N,N',N'-tetramethyl-p-phenylenediamine (TMPD), Fe(CN)6(4-), 1,1'-dimethyl-4,4'-bipyridinium dichloride (methyl viologen, MV(2+)) and Fe(CN)6(3-). In an argon-purged solution, ART reacts with solvated electrons (es(-)) with k=4.4 x 10(9) dm(3) mol(-1) s(-1) generating an absorption band rising in the ultraviolet region similar to the spectrum of the CH3(*)CHOH radical. The species originating from the reaction between ART and es(-) do not show any appreciable reactivity toward Fe(CN)6(4-), TMPD, MV(2+) and Fe(CN)6(3-). The experiments performed in the presence of ART and MV(2+) have provided strong support to the idea that the first species obtained from the addition of the electron, which is believed to occur at the endoperoxide group level, undergoes a rapid (k on the order of 10(8) s(-1) or higher) intramolecular rearrangement to give species, most likely carbon-centred radicals, that show some reactivity towards the methyl viologen radical cation (MV(*+)).


Asunto(s)
Artemisininas/efectos de la radiación , Sesquiterpenos/efectos de la radiación , Artemisininas/química , Radicales Libres , Oxidación-Reducción , Radiólisis de Impulso , Sesquiterpenos/química
15.
J Immunol Methods ; 174(1-2): 269-79, 1994 Sep 14.
Artículo en Inglés | MEDLINE | ID: mdl-8083532

RESUMEN

We have developed a method to generate immortalized phagocytic and dendritic cell clones from various mouse tissues such as spleen, thymus, brain and bone marrow. The clones were phenotypically characterized and shown to retain the ability to respond to immune or inflammatory signals, e.g., IFN-gamma. Functional cytokine activity and nitric oxide production were maintained in activated macrophages, microglial and dendritic cell clones. Immune functions, such as antigen presentation was exhibited by all clones whereas tissue-specific properties such as the ability to respond to corticotropin-releasing hormone and produce beta-endorphin was shown in microglial cell clones but not in macrophage cell clones, indicating that heterogeneity of cells of the mononuclear-phagocytic lineage can be maintained in vitro after the immortalization procedure. Moreover, the continuous proliferation of the clones could be inhibited by various stimuli and further differentiation of the cells could be achieved in vitro.


Asunto(s)
Transformación Celular Viral , Células Dendríticas/citología , Fagocitos/citología , Animales , Células Presentadoras de Antígenos/citología , Diferenciación Celular/efectos de los fármacos , División Celular , Células Clonales , Inmunofenotipificación , Inflamación/patología , Interferón gamma/farmacología , Ratones , Ratones Endogámicos , Óxido Nítrico/análisis , Retroviridae , Factor de Necrosis Tumoral alfa/biosíntesis , betaendorfina/metabolismo
16.
Neuromuscul Disord ; 2(4): 277-83, 1992.
Artículo en Inglés | MEDLINE | ID: mdl-1483054

RESUMEN

This paper reports the results of a clinical, genetic and histopathological study of 19 patients belonging to a large inbred Palestinian family living in Um-El-Fahem, a town located in Israel, which is solely inhabited by Arabs. Their custom of marrying only among relatives has kept the genetic homogeneity of the families intact. There were ten cases of congenital muscular dystrophy (CMD) and nine cases of adult limb-girdle muscular dystrophy (LGMD) belonging to two generations of the same family. Both forms showed autosomal recessive inheritance. The patients with congenital muscular dystrophy had generalized muscular weakness and hypotonia at birth without arthrogryposis or CNS involvement and then had a relatively benign evolution with stabilization of the clinical picture at different ages and variable degree of severity. Muscle biopsy showed a dystrophic pattern. The other nine patients presented with the picture of adult limb-girdle muscular dystrophy but with an unusual tendency to the stabilization of symptoms.


Asunto(s)
Distrofias Musculares/genética , Adolescente , Adulto , Anciano , Consanguinidad , Electromiografía , Femenino , Humanos , Israel , Masculino , Persona de Mediana Edad , Distrofias Musculares/etnología , Distrofias Musculares/patología , Distrofias Musculares/fisiopatología , Linaje
17.
Neuromuscul Disord ; 11(1): 20-6, 2001 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-11166162

RESUMEN

The limb-girdle muscular dystrophies are a group of inherited neuromuscular disorders which are clinically and genetically heterogeneous. We have been able to carry out a follow-up study on 10 patients from a large Palestinian family with a confirmed mutation in the dysferlin gene. These patients have been followed for more than 23 years since the onset of the disease. They all had normal developmental milestones. The onset of the disease was usually in the second decade, more rarely in the third and fourth decades. The first symptoms were difficulty with running and climbing stairs. Patients showed a distinct type of gait due to the unique pattern of muscle involvement which was characterised by early involvement of the posterior muscle compartment of the thighs and legs (hamstrings, adductors, gastrocnemius and soleus). The shoulder and upper limb musculature became involved later, especially supra and infraspinatus and biceps. In the early stages of disease these patients may clinically show only proximal lower limb-girdle muscle weakness; however, the use of muscle imaging techniques were very important, always detecting in these patients also distal lower limb muscle involvement, so that the pattern of muscle involvement found in dysferlin deficiency may not strictly conform to the definition of limb-girdle muscular dystrophy. The pattern of muscular dystrophy is essentially uniform and has clearly distinct features (involving mainly the initial pattern of muscle involvement and the mode of gait) which differ significantly from the well reported clinical features associated with sarcoglycanopathy, calpainopathy and Miyoshi myopathy.


Asunto(s)
Mutación del Sistema de Lectura/genética , Proteínas de la Membrana , Proteínas Musculares/genética , Distrofias Musculares/patología , Distrofias Musculares/fisiopatología , Adolescente , Adulto , Anciano , Progresión de la Enfermedad , Disferlina , Femenino , Estudios de Seguimiento , Trastornos Neurológicos de la Marcha/genética , Trastornos Neurológicos de la Marcha/fisiopatología , Homocigoto , Humanos , Masculino , Persona de Mediana Edad , Trastornos del Movimiento/genética , Trastornos del Movimiento/fisiopatología , Debilidad Muscular/genética , Debilidad Muscular/fisiopatología , Distrofias Musculares/genética , Fenotipo , Postura/fisiología
18.
Neuromuscul Disord ; 6(6): 483-90, 1996 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-9027859

RESUMEN

This study reports on a detailed clinical, electrophysiological, muscle computed tomography (CT) and laboratory investigation carried out on five families with definite linkage to chromosome 2p. Some clinical and laboratory features were common to most of the patients, such as the very high serum creatine kinase (CK) levels (mean 43.70 times the normal). The onset was most frequently in the late teens or early twenties with weakness and wasting of the pelvic girdle muscles. All patients had normal motor milestones and had not complained of any symptoms of muscle disease in early childhood. The clinical course was variable both between and within some families, but was most often slowly progressive. Some variability in the pattern of muscle involvement between the different families has also been observed.


Asunto(s)
Cromosomas Humanos Par 2 , Etnicidad/genética , Ligamiento Genético , Músculo Esquelético/fisiopatología , Distrofias Musculares/genética , Adolescente , Adulto , Creatina Quinasa/sangre , Progresión de la Enfermedad , Extremidades , Femenino , Contractura de la Cadera , Humanos , Masculino , Músculo Esquelético/patología , Distrofias Musculares/patología , Fenotipo , Pruebas de Función Respiratoria
19.
Chemistry ; 6(9): 1629-45, 2000 May 02.
Artículo en Inglés | MEDLINE | ID: mdl-10839180

RESUMEN

The cyclophane-type molecular dyads 1 x 2H and 1 x Zn, in which a doubly bridged porphyrin donor adopts a close, tangential orientation relative to the surface of a fullerene acceptor, were prepared by Bingel macrocylization. The porphyrin derivatives 2 x 2H and 2 x Zn with two appended, singly linked C60 moieties were also formed as side products. NMR investigations revealed that the latter compounds strongly prefer conformations with one of the carbon spheres nesting on the porphyrin surface, thereby taking a similar orientation to that of the fullerene moiety in the doubly bridged systems. Cyclic voltammetric measurements showed that the mutual electronic effects exerted by the fullerene on the porphyrin and vice versa are only small in all four dyads, despite the close proximity of the donor and acceptor components. The steady-state and time-resolved absorption and luminescence properties of 1 x Zn and 2 x Zn were investigated in toluene solution and it was shown that, upon light excitation, both the porphyrin- and the fullerene-centered excited states are deactivated to a lower-lying CT state, emitting in the IR spectral region (lambda max = 890 and 800 nm at 298 and 77 K, respectively). In the more polar solvent benzonitrile, this CT state is still detected but, owing to its very low energy (below 1.4 eV), is not luminescent and shorter-lived than in toluene. The remarkable observation of similar photophysical behavior of 1 x Zn and 2 x Zn suggests that a tight donor-acceptor distance cannot only be established in doubly bridged cyclophane-type structures but also in singly bridged dyads, by taking advantage of favourable fullerene-porphyrin ground-state interactions.

20.
J Neurol ; 234(6): 430-2, 1987 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-3655848

RESUMEN

In 1981 a report appeared of a patient with Duchenne muscular dystrophy associated with dwarfism caused by growth hormone deficiency, in whom the muscular disease was unusually benign. The authors suggested that the benign course might be related to the growth hormone deficiency and dwarfism. Other authors later supported this idea, having observed that in dystrophic mice and hamsters with congenital and experimentally induced pituitary dwarfism, respectively, pathological expressions of the dystrophy were markedly reduced. In this paper one case of Becker and one of limb-girdle dystrophy, each associated with short stature and growth hormone deficiency are described. In these cases the disease did not have a particularly benign course. It is concluded that caution is necessary, at least in certain cases, before an association between reduced muscular growth and the dystrophic process can be assumed.


Asunto(s)
Enanismo Hipofisario/complicaciones , Distrofias Musculares/complicaciones , Adolescente , Adulto , Enanismo Hipofisario/tratamiento farmacológico , Femenino , Hormona del Crecimiento/uso terapéutico , Humanos , Masculino , Distrofias Musculares/genética
SELECCIÓN DE REFERENCIAS
DETALLE DE LA BÚSQUEDA