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Biomedical subjects

J C Williams

Publications and source records attributed to J C Williams.

At least 181 records · Page 10Linked to original sources
PubMed1992

Chromosomal localization of the human glycoasparaginase gene to 4q32-q33.

Glycoasparaginase cleaves the N-glycosidic linkage between asparagine and N-acetylglucosamine in the degradation of glycoproteins. In humans, a deficient activity of glycoasparaginase results in accumulation of glycoasparagines, causing the lysosomal storage disease aspartylglycosaminuria. Recombinant plasmid containing the cDNA insert encoding human glycoasparaginase was used to localize the enzyme to chromosome 4q32-q33 by in situ hybridization to metaphase chromosomes prepared from normal human lymphocytes.

Aspartylglucosylaminase↗
PubMed1992

Paternal origin of 11p15 duplications in the Beckwith-Wiedemann syndrome. A new case and review of the literature.

A boy suffering from the Beckwith-Wiedemann syndrome (BWS) was found to have partial trisomy of the short arm of chromosome 11 [46,XY,der(5)t(5;11)(p15.2;p14)]. Both his parents were phenotypically normal, but his father carried a balanced translocation between chromosomes 5 and 11 [46,XY,t(5;11)(p15.2;p14)]. DNA analysis of polymorphic markers on 11p15 confirmed the paternal origin of the duplicated material in the child. This case is the sixth report of paternal duplication of 11p15 in BWS. These results are discussed in relation to the possible role of genomic imprinting in BWS and in Wilms' tumor.

Beckwith-Wiedemann Syndrome↗
PubMed1992

Efficacy of abamectin against natural infections of gastrointestinal nematodes and lungworm of cattle with special emphasis on inhibited, early fourth stage larvae of Ostertagia ostertagi.

The anthelmintic efficacy of abamectin (avermectin B1) was evaluated against gastrointestinal nematodes, including Ostertagia ostertagi inhibited larvae and lungworm, in yearling crossbred beef heifers during late spring. The calves were grazed on contaminated pasture for 10 weeks and then held under conditions free of nematode infection for 3 weeks prior to allotment and treatment on 5 June. Thirteen calves were randomly assigned to two groups of six by restricted randomization on body weights; the extra lightest calf was assigned to the non-treated control group. Group 1 calves were treated with abamectin at 200 micrograms kg-1 body weight by s.c. injection and Group 2 calves were not treated; all were killed at 14 days after treatment. Ostertagia ostertagi was present in all controls; arithmetic mean numbers of adults, developing fourth stage larvae (L4) and inhibited EL4 were 7683, 605 and 36,102, respectively. Other nematode genera present in controls in sufficient numbers for the experiment were Haemonchus placei adults, Trichostrongylus axei adults, Cooperia spp. adults, Oesophagostomum radiatum adults, Bunostomum phlebotomum adults, Dictyocaulus viviparus adults and E5 (immature adults). Abamectin was highly effective (consistently greater than 99% efficacy and P less than 0.05) in removing all nematodes present in treated calves as represented in non-treated controls, including the primary target of Ostertagia ostertagi inhibited EL4. The lowest efficacy was 93.8%, against D. viviparus E5.

Animals↗
PubMed1992

Evaluation of ivermectin against experimental infections of Haemonchus contortus and Trichostrongylus colubriformis in goats.

Fourteen nematode parasite-naïve kids were given a mixed larval inoculum (approximately 17,250 infective larvae) via stomach tube. Twenty-two days after infection, the kids were randomly assigned to control and treatment groups of seven animals each. Mean eggs per gram for control and treatment groups were 4304 and 5315, respectively. Kids in the treatment group were given ivermectin (0.2 mg kg-1) administered by subcutaneous injection. At necropsy, the mean numbers of Haemonchus contortus and Trichostrongylus colubriformis for control and treatment groups were 2259 and 0, and 3033 and 773, respectively. This reduction was significant (P less than 0.05) for both species, resulting in an efficacy of 100.0% and 74.5%, respectively. The lower efficacy against T. colubriformis may be a sign of resistance, a reduced effectiveness due to route of administration, or a higher dose may be required with subcutaneous administration, as has been observed previously.

Animals↗
PubMed1992

Effect of fenbendazole molasses supplement block treatment on nematode infection and subsequent weight gain of weanling beef calves.

Two trials were conducted to evaluate the effect of fenbendazole molasses supplement block treatment on weight gain of weanling beef calves. An initial (November) oral fenbendazole (5 mg kg-1) treatment was administered to all animals in both trials. In Trials 1 and 2, respectively, two fenbendazole molasses supplement block treatments were administered 6 (6.4 mg kg-1) and 16 (7.0 mg kg-1) weeks later and 7 (3.4 mg kg-1) and 14 (3.8 mg kg-1) weeks later. During the treatment periods, calves were maintained on dormant bermuda grass pasture, supplemented with hay and protein/mineral blocks. The first block treatment was administered approximately midway through the supplement period to suppress the infection level when nutrition was marginal. The second treatment was administered just prior to ryegrass grazing. Trial 1 and 2 treatments resulted in reducing the infection level, as indicated by reduced mean fecal egg counts. Treatment group mean fecal egg counts remained lower than control group mean counts throughout each trial. In Trial 1, the treatment group gained 29.5 kg more than the control group. In Trial 2, the control group gained 15.5 kg more than the treatment group. Fecal egg count data indicated that the infection level during Trial 1 was higher than during Trial 2. This difference could influence weight gain.

Administration, Oral↗
PubMed1992

Efficacy of Cydectin moxidectin 1% injectable against experimental infections of Dictyocaulus viviparus and Bunostomum phlebotomum superimposed on natural gastrointestinal infections in calves.

Twenty male Holstein calves averaging 105 kg in weight and naturally infected with gastrointestinal nematodes and small numbers of lungworm and hookworm, were given experimental infections with the two latter species to provide adult and larval stages for anthelmintic evaluation. Following random allotment, one group of 10 calves was injected subcutaneously with moxidectin at a dosage of 0.2 mg kg-1 of body weight. A second group of 10 was injected subcutaneously with unmedicated blank vehicle at a dosage of 1 ml per 50 kg of body weight. Fecal samples were examined before treatment and at 7 and 13 days after treatment. The 20 calves were necropsied for worm recovery at 13 and 14 days after treatment. All calves were positive for lungworm and hookworm on the treatment date. Treatment was 100% effective in elimination of hookworm eggs and lungworm larvae and 99.9% in reducing total egg counts at both 7 and 13 days after treatment. Moxidectin was 100% effective (P less than 0.01) in eliminating the following 11 species of nematodes. Dictyocaulus viviparus mature and immature adults (E5), Bunostomum phlebotomum adults and L4, Ostertagia ostertagi adults and early L4, Ostertagia lyrata adult males, Haemonchus placei adults. Trichostrongylus axei adults, Cooperia spp., including Cooperia punctata, Cooperia spatulata, and Cooperia pectinata adults, Oesophagostomum radiatum adults and Trichuris discolor adults. No adverse reactions to moxidectin treatment were observed.

Animals↗
PubMed1992

Immunogenicity and toxicity of lipopolysaccharide from Francisella tularensis LVS.

Lipopolysaccharide (LPS) from the live vaccine strain of Francisella tularensis (F. tularensis LVS) was isolated and purified. The LPS did not stimulate lymphocytes from previously tularaemia-vaccinated individuals or lymphocytes from non-primed individuals. However, serum antibodies from tularaemia vaccinees reacted with the LPS whereas virtually no reactivity was found with antibodies from individuals not exposed to F. tularensis LVS. Antibodies of immunoglobulin class M displayed the antibody reactivity predominantly. The LPS failed to induce the mononuclear cell-derived cytokine interleukin-1 and only low levels of tumour necrosis factor were detected. Furthermore, no LPS endotoxin properties were found in galactosamine-treated mice or in the Limulus amoebocyte lysate assay. From these results it can be concluded that F. tularensis LVS possesses a lipopolysaccharide-like molecule, which does not exhibit properties of a classical endotoxin.

Animals↗
PubMed1992

A Coxiella burnetti repeated DNA element resembling a bacterial insertion sequence.

A DNA fragment located on the 3' side of the Coxiella burnetii htpAB operon was determined by Southern blotting to exist in approximately 19 copies in the Nine Mile I genome. The DNA sequences of this htpAB-associated repetitive element and two other independent copies were analyzed to determine the size and nature of the element. The three copies of the element were 1,450, 1,452, and 1,458 bp long, with less than 2% divergence among the three sequences. Several features characteristic of bacterial insertion sequences were discovered. These included a single significant open reading frame that would encode a 367-amino-acid polypeptide which was predicted to be highly basic, to have a DNA-binding helix-turn-helix motif, to have a leucine zipper motif, and to have homology to polypeptides found in several other bacterial insertion sequences. Identical 7-bp inverted repeats were found at the ends of all three copies of the element. However, duplications generated by many bacterial mobile elements in the recipient DNA during insertion events did not flank the inverted repeats of any of the three C. burnetii elements examined. A second pair of inverted repeats that flanked the open reading frame was also found in all three copies of the element. Most of the divergence among the three copies of the element occurred in the region between the two inverted repeat sequences in the 3' end of the element. Despite the sequence changes, all three copies of the element have retained significant dyad symmetry in this region.

Amino Acid Sequence↗
PubMed1992

Cell-mediated and humoral immune responses induced by scarification vaccination of human volunteers with a new lot of the live vaccine strain of Francisella tularensis.

Tularemia is a disease caused by the facultative intracellular bacterium Francisella tularensis. We evaluated a new lot of live F. tularensis vaccine for its immunogenicity in human volunteers. Scarification vaccination induced humoral and cell-mediated immune responses. Indications of a positive immune response after vaccination included an increase in specific antibody levels, which were measured by enzyme-linked immunosorbent and immunoblot assays, and the ability of peripheral blood lymphocytes to respond to whole F. tularensis bacteria as recall antigens. Vaccination caused a significant rise (P less than 0.05) in immunoglobulin A (IgA), IgG, and IgM titers. Lymphocyte stimulation indices were significantly increased (P less than 0.01) in vaccinees 14 days after vaccination. These data verify that this new lot of live F. tularensis vaccine is immunogenic.

Antibody Formation↗
PubMed1992

Otolaryngologic manifestations of the mucopolysaccharidoses.

A retrospective review of 45 children with mucopolysaccharidoses was performed to determine the frequency of complications related to the head and neck. In this series, every patient had at least one complication involving the head and neck region, and in over half, operative intervention by the otolaryngologist was required. Upper airway obstruction occurred in 17 (38%) and necessitated a tracheostomy in 7 (16%). Cervical spine instability occurred in 8 (18%), making airway management difficult. Recurrent respiratory infections occurred in 17 (38%), and chronic recurrent middle ear effusions were noted in 33 (73%). This review demonstrates that children afflicted with the mucopolysaccharidoses frequently have otolaryngologic-related complications that are common throughout their life span and often the primary management issue in their continuing care. The otolaryngologic management of these patients is outlined based on the results of this study and review of the relevant literature.

Adolescent↗
PubMed1992

Efficacy of ivermectin delivered from a sustained-release bolus against inhibited early fourth-stage larvae of Ostertagia ostertagi and other nematodes in cattle.

The anthelmintic efficacy of ivermectin (IVM) delivered from a sustained-release (SR) bolus was evaluated against natural infections with gastrointestinal tract nematodes in 12 crossbred beef heifers in spring. The 12 calves were randomly allotted to 2 groups of 6 calves each. Group-1 calves were treated with an SR bolus designed to deliver 8 mg of ivermectin/d. Group-2 calves were nontreated controls. Cattle groups were kept in separate concrete-floored pens (grass hay nutrition) and slaughter was performed at 35 days after treatment. Fecal egg counts for group-1 calves remained zero after treatment, except for detection of less than 1 egg/g of feces in 1 calf at the time of slaughter; counts in nontreated calves increased. Mean and range of Ostertagia ostertagi inhibited larvae in nontreated calves were 27,093 and 10,622 to 56,368, respectively. Efficacy of the IVM SR bolus was 100% against O ostertagi developing fourth-stage larvae (L4) and inhibited early L4, Haemonchus placei adults, Cooperia punctata and C spatulata adult males, Cooperia spp adult females, Cooperia spp L4, Trichostrongylus colubriformis adults, Bunostomum phlebotomum adults, and Oesophagostomum radiatum adults. Efficacy for O ostertagi and T axei adults was 99.9%. Numbers of nontreated calves infected with C pectinata adult males and Oes radiatum L4 were too low to evaluate efficacy. Calves treated with the IVM bolus gained 10.2 kg, whereas nontreated calves lost 1.8 kg. Abomasal lesions were clearly greater in nontreated calves on the basis of index comparisons of abomasal weight and total live weight and gross pathologic features.

Abomasum↗
PubMed1991

The primary structure of cytochrome c-554 from the green photosynthetic bacterium Chloroflexus aurantiacus.

The complete nucleotide sequence of the cytochrome c-554 gene from the green photosynthetic bacterium Chloroflexus aurantiacus has been determined. The derived amino acid sequence showed that the cytochrome precursor protein consists of 414 residues and contains 4-Cys-X-X-Cys-His- heme binding motifs. The only regions of the cytochrome c-554 sequence that were found to be significantly similar to the sequences of cytochromes from other organisms were the heme binding sites. The highest similarity was found with the heme binding segments in the four-heme reaction center cytochrome subunit from the purple photosynthetic bacterium Rhodopseudomonas viridis. The importance of this similarity for the evolutionary relationship between Chloroflexus and the purple bacteria is discussed.

Amino Acid Sequence↗
PubMed1991

Increased analytical precision in the hollow cathode discharge emission source by improved discharge current control.

A significant improvement in the precision of the hollow cathode as an emission source is reported. Precision of 1% or less has been observed several times over periods of several hours. An average long-term stability of 4.3% for Ll and 6.0% for Na in the emission signal from microsamples (less than 50 nL) deposited in the hollow cathode discharge source is reported. The improved precision is attributed primarily to the introduction of electronics that hold the discharge current more nearly constant and to the shielding of all wiring to the source from the power supply. A current-controlled switch that is capable of driving a hollow cathode discharge in either dc or pulsed mode is described. This switch is capable of generating current pulses as short as 2 microseconds through a resistive load and greatly improves the discharge stability and repeatability at turn-on in both the dc and pulsed modes. The characteristics of pulses produced by this switch are presented; however, analytical performance is reported only for the dc mode. Temporal current plots are presented for the new instrumentation and compared to plots taken with commonly used current-controlled power supplies. Instrumentation, operation, and sample preparation procedures are described. Typical temporal profiles of the emission signal from microsamples deposited in AI and stainless steel hollow cathodes are given.

Sodium↗
PubMed1991

Aspartylglycosaminuria in the Finnish population: identification of two point mutations in the heavy chain of glycoasparaginase.

Aspartylglycosaminuria is an inherited lysosomal storage disease caused by deficiency of glycoasparaginase (EC 3.5.1.26) and occurs with higher frequency among Finns than other populations. We have purified human glycoasparaginase and determined about 90% of the amino acid sequence of its light subunit and greater than 70% of that of its heavy subunit by Edman degradation and mass spectrometry. Additional sequence data were obtained from the cloning and subsequent nucleotide analysis of a cDNA corresponding to the normal human glycoasparaginase gene. The enzyme is encoded by a single mRNA as a single polypeptide that is posttranslationally processed to generate the subunits and is glycosylated. After preparing first-strand cDNA from leukocyte and fibroblast total RNA, we used the polymerase chain reaction to amplify the glycoasparaginase cDNA of eight Finnish aspartylglycosaminuria patients. We demonstrate that the Finnish patients' mRNA sequence differed from the normal sequence by two single-base changes six nucleotides apart from one another in the heavy chain of glycoasparaginase. The first change resulted in the replacement of arginine by glutamine (R161Q), whereas the second change resulted in a cysteine to serine substitution (C163S). Both mutations resulted in novel restriction endonuclease sites and were present in all eight Finnish aspartylglycosaminuria patients originating from different pedigrees, but they were absent from Finnish and non-Finnish controls and a non-Finnish case of aspartylglycosaminuria. These results indicate molecular homogeneity in aspartylglycosaminuria alleles in the Finnish population.

Amino Acid Sequence↗
PubMed1991

Glycosaparaginase from human leukocytes. Inactivation and covalent modification with diazo-oxonorvaline.

The apparent active site of human leukocyte glycoasparaginase (N4-(beta-acetylglucosaminyl)-L-asparaginase EC 3.5.1.26) has been studied by labeling with an asparagine analogue, 5-diazo-4-oxo-L-norvaline. Glycoasparaginase was purified 4,600-fold from human leukocytes with an overall recovery of 12%. The purified enzyme has a Km of 110 microM, a Vmax of 34 mumol x l-1 x min-1, and a specific activity of 2.2 units/mg protein with N4-(beta-N-acetylglucosaminyl)-L-asparagine as substrate. The carbohydrate content of the enzyme is 15%, and it exhibits a broad pH maximum between 7 and 9. The 88-kDa native enzyme is composed of 19-kDa light (L) chains and 25-kDa heavy (H) chains and it has a heterotetrameric structure of L2H2-type. The glycoasparaginase activity decreases rapidly and irreversibly in the presence of 5-diazo-4-oxo-L-norvaline. At any one concentration of the compound, the inactivation of the enzyme is pseudo-first-order with time. The inhibitory constant, K1, is 80 microM and the second-order rate constant 1.25 x 10(3) M-1 min-1 at pH 7.5. The enzyme activity is competitively protected against this inactivation by its natural substrate, aspartylglucosamine, indicating that this inhibitor binds to the active site or very close to it. The covalent incorporation of [5-14C]diazo-4-oxo-L-norvaline paralleled the loss of the enzymatic activity and one inhibitor binding site was localized to each L-subunit of the heterotetrameric enzyme. Four peptides with the radioactive label were generated, purified by high performance liquid chromatography, and sequenced by Edman degradation. The sequences were overlapping and all contained the amino-terminal tripeptide of the L-chain. By mass spectrometry, the reacting group of 5-diazo-4-oxo-L-norvaline was characterized as 4-oxo-L-norvaline that was bound through an alpha-ketone ether linkage to the hydroxyl group of the amino-terminal amino acid threonine.

Amino Acid Sequence↗
PubMed1991

N-acetylglutamate synthetase deficiency: clinical and laboratory observations.

Two male siblings presented in the first 6 weeks of life with emesis, diarrhoea, metabolic acidosis and lethargy. A male sibling had previously died at 14 months of age from liver failure of unknown aetiology. Both of the current cases had mild hyperammonaemia with normal orotic acid, organic acid and argininosuccinic acid levels. Citrulline and arginine levels were normal or mildly decreased. One of the brothers was biopsied and had no detectable N-acetylglutamate synthetase activity and normal values for other enzymes of the urea cycle in liver. Treatment with a low-protein diet and sodium benzoate/sodium phenylacetate resulted in near normal blood ammonia levels, except during viral illness. Subsequent neurological development has been normal to mildly delayed. These patients differ from those previously described with N-acetylglutamate synthetase deficiency in that their presentation and subsequent course were relatively benign.

Acetyltransferases↗
PubMed1991

Efficacy of albendazole, levamisole and fenbendazole against gastrointestinal nematodes of cattle, with emphasis on inhibited early fourth stage Ostertagia ostertagi larvae.

An experiment was conducted to compare the anthelmintic efficacy of albendazole (ABZ), levamisole (LEV) and fenbendazole (FBZ) against inhibited early fourth stage larvae (EL4) of Ostertagia ostertagi during April in Louisiana. Forty cross-bred beef heifers (average weight 165 kg) were acquired during winter and grazed on pastures contaminated with O. ostertagi and other nematodes until early April. The cattle were weighed and randomly allotted into four groups of ten animals on 6 April and treatments were administered on 7 April. Experimental groupings were: Group 1, non-treated controls; Group 2, ABZ by oral drench at 10 mg kg-1; Group 3, LEV by topical, pour-on administration to back midline at 10 mg kg-1; Group 4, FBZ by oral drench at 10 mg kg-1. Equal numbers of cattle from each group were slaughtered daily between 10 and 13 days after treatment. Mean numbers of O. ostertagi developmental stages present in untreated controls were: adults, 13,714; developing L4 (DL4), 6487; inhibited EL4, 21,719. The mean percentage of inhibited EL4 was 51.8. Smaller numbers of Haemonchus placei, Trichostrongylus axei and Cooperia spp. were recovered uniformly in control cattle. Percentage reduction values for the three compounds against O. ostertagi adults, DL4 and EL4, respectively, were: ABZ, 99.0, 95.3, 84.9; LEV, 1.0, 21.8, 32.1; FBZ, 99.2, 97.2, 97.5. Differences between ABZ and LEV EL4 counts were not significant, but in all other cases worm counts in ABZ and FBZ-treated cattle were significantly lower (P less than or equal to 0.05) than in LEV-treated and control cattle. Both ABZ and FBZ were 98-100% effective against Haemonchus adults and L4, T. axei adults, and Cooperia spp. adults and L4. LEV was 100% effective against Haemonchus adults and L4, 85.6% against T. axei, and 94.6% and 89.59% effective against Cooperia spp. adults and L4, respectively.

Abomasum↗