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Biomedical subjects

J Oliver

Publications and source records attributed to J Oliver.

At least 127 records · Page 7Linked to original sources

Ehlers-Danlos syndrome type VII: a single base change that causes exon skipping in the type I collagen alpha 2(I) chain.

We have examined the procollagens and collagens produced by skin fibroblasts from a patient with Ehlers-Danlos syndrome type VII. The patient was heterozygous for an abnormal alpha 2(I) chain migrating with the approximate size of pN alpha 2(I) chains after pepsin digestion. Peptide mapping suggested that the abnormality was located at the amino-terminus of the alpha 2(I) chain. Quantitative analysis of the alpha 2(I) mRNA indicated loss of the exon 6 sequences, and subsequent polymerase chain reaction amplification of cDNA demonstrated a deletion of the 54 bp of exon 6 from some of the alpha 2(I) mRNA. Analysis of genomic DNA from the patient revealed a single base change in one COL1A2 allele, substituting an A for a G as the first base of intron 6. This change mutates the obligate GT-dinulceotide splicing signal to AT and leads to exon skipping with splicing from exon 5 to exon 7. Loss of exon 6 sequences results in the loss of the procollagen-N-propeptidase cleavage site and a lysine residue that normally participates in covalent intermolecular crosslinking within collagen fibres.

Adult↗

Cockroach allergenic activity: analysis of commercial cockroach and dust extracts.

Previous investigations demonstrated that cockroach whole bodies and feces are important sources of allergens in the induction/exacerbation of bronchial asthma. The current study investigated different cockroach source materials, commercial extracts, and house dust extracts for cockroach allergenic activity. In general, extracts from four different sources of either American or German cockroaches contained similar amounts of allergenic activity by RAST inhibition. Three commercial American cockroach extracts compared by RAST inhibition had similar allergenic activity on an equal protein basis. Skin test results correlated house dust reactivity to both commercial and inhouse cockroach wholebody extracts and to fecal extracts. Six different samples of house dust obtained from vacuum cleaners in the New Orleans area and three commercially obtained house dust extracts contained varying quantities of cockroach allergenic activity by RAST inhibition. These studies demonstrate that commercial cockroach extracts vary in allergenic activity and that all house dust extracts tested contain cockroach allergens.

Allergens↗

Kinetic characterization of phosphofructokinase isolated from rat kidney cortex.

1. Phosphofructokinase from rat kidney cortex has been purified by affinity chromatography to a final specific activity of 15 units per mg of protein, measured at 25 degrees C and pH 8. 2. This lower spec. act., compared with that of the enzyme from other sources, shows the enzyme in proximal tubules to be less active, which would account for the main gluconeogenic role of these nephron sections. 3. The binding of fructose-6-phosphate to the enzyme is co-operative. ATP increases the Hill coefficient and produces a marked allosteric inhibition on the activity. 4. Fructose-2,6-bis-phosphate is a potent activator of the enzyme from this source. It reduces the Hill coefficient of the enzyme and the inhibition constant of ATP. A marked difference between this and the liver enzyme is that the activation is not co-operative.

Adenosine Triphosphate↗

Distribution of arrestin-like protein and beta-subunit of GTP-binding proteins in quail choroid plexuses.

Monoclonal antibodies (Mabs) directed against retinal arrestin (S-antigen) were used to detect and characterize this protein in choroid plexus (CP) of quails maintained during eight days, either under long-day photoperiods or in constant darkness. Immunocytochemistry and Western blotting confirmed the presence and the distribution of an arrestin-like protein in quail CP. Arrestin-like immunoreactivities in CP were compared with those obtained with Mabs to beta 36-subunit of G proteins (G beta), alpha-subunit of transducin and rhodopsin. Rhodopsin-like and transducin-like proteins could not be detected in choroidal cells, whereas intense positive reactions were observed with anti-G beta and anti-arrestin Mabs. The strongest immunoreactivities were found in choroidal ependymocytes of the lateral and IIIrd ventricles. In CP epithelial cells lining the IVth ventricle, very weak or no immunoreactivity could be detected with Mabs to arrestin, while Mab against G beta subunit always provided a positive reaction. In quails maintained in constant darkness, arrestin- and G beta-immunoreactivities of CP epithelial cells displayed changes in cellular distribution and intensity (decrease or disappearance of the immunoreactions). The strong arrestin-like immunoreaction located in the apical region of ependymocytes suggests the preferential association of the protein with choroidal microvilli and a possible role in cerebrospinal fluid production assumed by CP cells.

Animals↗

Distribution of pyruvate carboxylase along the rat nephron: an immunological and enzymatic study.

Antibodies against purified rat-kidney-cortex pyruvate carboxylase were raised in rabbits. These polyspecific antibodies recognize pyruvate carboxylase enzymes alone without cross reactivity with other carboxylases as detected by immunoblotting. The abundance of pyruvate carboxylase in the various renal fractions was measured by ELISA and its activity by the fixation of [14C]CO3H-. The results were corroborated by a combination of immunocytochemistry and transmission electron microscopy. A good correlation was found between the enzymatic activity and the quantity of enzyme contained in each fraction. The kidney-cortex pyruvate carboxylase was primarily located in proximal tubules, in accord with its important role in gluconeogenesis.

Animals↗

Substitution of cysteine for glycine at residue 415 of one allele of the alpha 1(I) chain of type I procollagen in type III/IV osteogenesis imperfecta.

We have examined the type I collagen in a patient with type III/IV osteogenesis imperfecta. Two forms of alpha 1(I) chain were produced, one normal and the other containing a cysteine residue within the triple helical domain of the molecule. Cysteine is not normally present in this domain of type I collagen. Peptide mapping experiments localised the mutation to peptide alpha 1(I)CB3 which spans residues 403 to 551 of the triple helix. Subsequent PCR amplification of cDNA covering this region followed by sequencing showed a G to T single base change in the GGC codon for glycine 415 generating TGC, the codon for cysteine. The effect of the mutation on the protein is to delay secretion from the cell, reduce the thermal stability of the molecule by 2 degrees C, and cause excessive post-translational modification of all chains in molecules containing one or more mutant alpha 1(I) chains. The clinical phenotype observed in this patient and the position of the mutation conform to the recent prediction of Starman et al that Gly----Cys mutations in the alpha 1(I) chain have a gradient of severity decreasing from the C-terminus to the N-terminus.

Alleles↗

Regulation of IgE responses to inhaled antigens: cellular mechanisms underlying allergic sensitization versus tolerance induction.

Previous research from our laboratory has established that the natural response of the respiratory mucosal immune system to inhaled allergens involves initial 'recognition' accompanied by transient low-level IgE production, followed by the development of protective immunological tolerance. Recent studies indicate that the crucial cellular events in this process occur at the level of the upper respiratory mucosa and in the local draining lymph nodes. Salient findings from ongoing studies, detailed below, include identification of a highly developed network of dendritic antigen-presenting cells within the airway epithelium which trap inhaled antigen, and definition of the surface phenotype of the suppressor T cells mediating tolerance as TcR gamma + delta +.

Administration, Oral↗

[Monoclonal antibodies obtained against G-protein epitopes: comparison of immunoreactions seen in the brain, retina and striated muscular tissue].

Monoclonal antibodies have been obtained against a purified fraction of brain G proteins containing the Gi alpha, G0 alpha, G beta, and G gamma subunits. After characterization, two monoclonal antibodies have been used to detect the cellular distribution of the two epitopes in neural, retinal and muscular tissues: ELISA, cross-dot and Western blot demonstrated that F.IV.5 is an anti-G beta antibody specific for the 36 kDa beta-subunit. ELISA, cross-dot and immunocytochemical distribution of the epitopes recognized by F.VII.9 suggested that this antibody recognizes epitopes which are also detected with polyclonal anti-G0 alpha antibodies. With both monoclonal antibodies, we confirmed that G proteins demonstrated a sub-membranous distribution as well as extensively cytoplasmic, axoplasmic or sarcoplasmic distributions in different cell types.

Animals↗

[Sclerosing treatment of esophageal varices].

The results of esophageal varices treatment in two groups of patients are shown. The first group consists of 351 patients who suffered an hemorrhage and were treated with sclerosing varices during the acute period or in the intervals of bleedings. The second group consists of 90 cirrhotic patients to whom a prophylactic treatment was administered. From 67 patients treated during acute hemorrhage 6 (9%) died due to hemorrhage, 6 (9%) due to hepatic failure and 1 (1.5%) due to sepsis. The 90 cirrhotic patients with prophylactic treatment were divided in 3 groups. In the first group of 30, 15 were sclerosized and 15 did not get treatment. From the second group of 32, 16 got propranolol and 16 no treatment. In the third group of 28, 14 got propranolol and were sclerosized and 14 were not treated. In all three groups with treatment hemorrhage ceased in a statistically significative manner. Surviving was the same in the treated and non-treated groups. Most patients died due to an hepatic failure.

Esophageal and Gastric Varices↗

Regulation of T-cell sensitization at epithelial surfaces in the respiratory tract: suppression of IgE responses to inhaled antigens by CD3+ Tcr alpha-/beta- lymphocytes (putative gamma/delta T cells).

Repeated exposure of Brown Norway rats to an aerosol of ovalbumin (OVA) induced a state of antigen-specific immunological tolerance, particularly in the IgE isotype. Tolerance was transferable to naive syngeneic animals by inoculation of splenic T cells from tolerant rats. Sequential depletion of tolerant spleen cells by sorting techniques prior to adoptive transfer, employing T-cell subset-specific monoclonal antibodies, indicated that the cells mediating tolerance were CD3+, CD4-, CD5+ and CD8+, but lacked alpha or beta chains in the T-cell receptor (TcR), suggesting that they may be part of the gamma/delta T-cell lineage. Consistent with this suggestion, the sorted population demonstrated considerable enrichment for TcR gamma chain-specific mRNA. As few as 2 x 10(3) cells are sufficient to adoptively transfer tolerance in 200-g adult rats in this model.

Administration, Inhalation↗

[Left ventricular perforation following percutaneous mitral valvuloplasty. Its emergency surgical correction in situ. Apropos a case].

Percutaneous mitral valvuloplasty is actually accepted as alternative to surgery for treatment of rheumatic mitral stenosis, although today it is not still free from troublesome complications like ventricular perforation. We present here a patient in which was performed a percutaneous mitral valvuloplasty and that developed a left ventricular perforation at the end of the procedure, with hyperacute cardiac tamponade, requiring an in situ surgical salvagement on an emergency basis, performing a successful repair of the ventricular rupture in the own catheterization laboratory.

Balloon Occlusion↗

Squamous cell carcinoma and lingual thyroid.

This report represents the first known case of squamous cell carcinoma associated with lingual thyroid tissue. Clinical examination of an exophytic mass at the base of tongue, CT imaging and a preoperative biopsy established a diagnosis of squamous cell carcinoma of the midline base of tongue. The therapeutic options and management of this carcinoma by surgical resection, irradiation or combined modalities were analyzed. The surgical option was chosen. This was accomplished via a midline labiomandibulo glossal split and primary repair of the wound. The final pathology report revealed a squamous cell carcinoma of the mucosa of the base of tongue directly over a benign lingual thyroid mass. Post-operative thyroid function studies were consistent with hypothyroidism and a thyroid scan confirmed the absence of thyroid tissue.

Aged↗

Immunoregulation of asthma: control of T-lymphocyte activation in the respiratory tract.

Allergic asthma results ultimately from inappropriate responses to non-pathogenic airborn antigens by the respiratory tract T-cell system. In order to understand how sensitization to inhaled antigens develops, it is necessary to precisely define the tissue microenvironments within which T-cells occur in the respiratory tract, and to analyse the factor(s) which regulate their local activation. This review focuses upon recent data on the distribution of T-cells and antigen presenting cells within the lung and airway tissues of man and experimental animals, and examines the available information on their responses to antigenic stimulation. We argue that in the steady state, respiratory tract T-cells are tightly regulated by a series of inherent and acquired immunosuppressive control mechanisms, which normally limit local T-cell activation to situations where antigenic exposure is accompanied by an inflammatory stimulus, such as the case with incoming pathogenic microorganisms.

Allergens↗

Association of antibodies against phospholipids with heart valve disease in systemic lupus erythematosus.

A prospective echocardiographic study was carried out on 132 consecutive patients with systemic lupus erythematosus (SLE) derived from three European university medical centres. The prevalence of valvular lesions in patients with SLE was 22.7% compared with 2.9% in a control group of 68 healthy volunteers. 50 SLE patients had antibodies against phospholipids. The prevalence of valve vegetations (8/50 [16%]) and of mitral regurgitation (19/50 [38%]) was significantly higher among the SLE patients with antiphospholipids than among those without (1 and 10/82 [1.2% and 12%], respectively). During follow-up of the patients with valvular lesions, haemodynamically significant clinical valve disease developed in 6 but surgery was required in only 1; 9 had cerebrovascular occlusions; and 7 died, although no death was due directly to the cardiac involvement. Thus, valvular heart disease, particularly affecting the mitral valve, is common in patients with SLE, and the presence of antibodies against phospholipids is associated with a higher prevalence of valvular abnormalities in these patients.

Adolescent↗

Stimulation of mitochondrial pyruvate transport in rat renal cortex by phenylephrine.

Phenylephrine effect on liver and kidney cortex mitochondrial pyruvate concentration was investigated. While in liver the alpha 1-adrenergic agent produced a decrease in pyruvate content, a significant increase was observed in kidney, even in the presence of 0.5 mM alpha-cyano-4-hydroxy-cinnamate. These changes were not observed when pyruvate was formed by intramitochondrial transamination of alanine, suggesting a role for the pyruvate transport across mitochondrial membranes in the regulation of mitochondrial pyruvate metabolism in kidney cortex. This was corroborated measuring the phenylephrine effect on pyruvate carboxylation.

Alanine↗

Prolonged changes in plasma concentrations of catecholamine metabolites following a single infusion of an MPTP analog.

The magnitude and duration of effects of a single intravenous injection of 4'-amino MPTP, an analogue of the dopamine neurotoxin, MPTP, on plasma levels of catechols and normetanephrine were examined in conscious dogs. Plasma samples were collected prior to treatment with intravenous saline or 4'-amino MPTP.2HCl (22.5 mg/kg) and at weekly intervals for six weeks following treatment. Saline treatment had no effect on plasma levels of any of the measured compounds. Following 4'-amino MPTP, plasma DHPG fell to 14% of the pre-injection value and remained decreased for the full 6-week test period, with partial recovery by week 6 to 42% of the pre-injection value. Plasma DOPAC levels fell to 28% of pre-injection values 1 week after treatment with 4'-amino MPTP and showed no evidence of recovery during the 6-week test period. Plasma DOPA fell to 58% of the pre-injection level, while concentrations of the catecholamines NE, EPI, and DA were generally unaffected. The plasma concentration of the O-methylated NE metabolite, normetanephrine, was also unchanged by 4'-amino MPTP treatment. There were no differences in the concentrations of DA, NE or EPI within the adrenal medulla between saline and 4'-amino MPTP treated groups. This pattern of changes in plasma levels of catechols, which is consistent with presynaptic inhibition of MAO within sympathetic terminals, may be a useful indicator of exposure to MPTP-like neurotoxins.

1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine↗