Your browser doesn't support javascript.
loading
Show: 20 | 50 | 100
Results 1 - 20 de 46
Filter
1.
Saudi J Biol Sci ; 29(12): 103453, 2022 Dec.
Article in English | MEDLINE | ID: mdl-36204726

ABSTRACT

The purpose of the current research was to determine the impact of various biochar supplements on nutrient digestibility and growth performance of Catla catla fingerlings fed on Moringa oleifera seed meal (MOSM) based diet. An experiment with 90 days of feeding was conducted to investigate the efficacy of biochar obtained from different sources such as parthenium, farmyard manure, poultry waste, vegetable waste, and corncob waste at 2 mg/kg. There were15 fingerlings in each tank in the triplicate set of tanks. Fingerlings were fed at the rate of 5 % of their live wet weight. From each tank, feces were collected twice daily and stored to determine nutrient digestibility. Results showed that poultry waste biochar (test diet-IV) at 2 mg/kg was the best source to significantly (P < 0.05) improve weight gain % (256.58 %) and feed conversion ratio (1.19) than the other biochar sources and control diet. Furthermore, in terms of nutrient digestibility, optimum fat (81.90 %), protein (75.92 %), and gross energy (74.84 kcalg-1) values were obtained by using the same type of biochar (poultry waste). Conclusively, among all the five biochar sources, poultry waste biochar proved to be the best one, improving fish body performance.

2.
Article in English | MEDLINE | ID: mdl-36078326

ABSTRACT

With the growing scarcity of water, the remediation of water polluted with heavy metals is the need of hour. The present research work is aimed to address this problem by adsorbing heavy metals ions (Pb (II) and Cr (VI)) on modified graphene oxide having an excess of carboxylic acid groups. For this, graphene oxide (GO) was modified with chloroacetic acid to produce carboxylated graphene oxide (GO-COOH). The successful synthesis of graphene oxide and its modification has been confirmed using Fourier transform infrared spectroscopy (FT-IR), Raman spectroscopy, X-ray Diffraction (XRD), Scanning electron microscopy (SEM), Energy Dispersive X-ray Analysis (EDX) and Transmission electron microscopy (TEM). The increase in surface area of graphene oxide after treatment with chloroacetic acid characterized by BET indicated its successful modification. A batch experiment was conducted to optimize the different factors affecting adsorption of both heavy metals on GO-COOH. After functionalization, we achieved maximum adsorption capacities of 588.23 mg g-1 and 370.37 mg g-1 for Pb and Cr, respectively, by GO-COOH which were high compared to the previously reported adsorbents of this kind. The Langmuir model (R2 = 0.998) and Pseudo-second-order kinetic model (R2 = 0.999) confirmed the monolayer adsorption of Pb and Cr on GO-COOH and the chemisorption as the dominant process governing adsorption mechanism. The present work shows that the carboxylation of GO can enhance its adsorption capacity efficiently and may be applicable for the treatment of wastewater.


Subject(s)
Graphite , Metals, Heavy , Water Pollutants, Chemical , Adsorption , Carboxylic Acids , Graphite/chemistry , Kinetics , Lead , Spectroscopy, Fourier Transform Infrared , Water/chemistry , Water Pollutants, Chemical/analysis
3.
Transl Anim Sci ; 5(4): txab154, 2021 Oct.
Article in English | MEDLINE | ID: mdl-34859198

ABSTRACT

There has always been a debate about the acceptability of stunning methods for preparing Halal slaughtered meat. Throughout the last few decades, stunning methods have become acceptable for Halal slaughtering due to an increasing majority of Muslim countries issuing Fatwas (religious rulings) that approve of stunning methods for the Halal slaughtering of food animals. With an increasing Muslim population worldwide, Halal meat provision is important for Muslims both economically and ethically. Moreover, there have been concerns regarding traditional Halal slaughter without the use of stunning from the standpoint of the animal's welfare. This article reviews the different stunning methods available and the associated processing practices, addressing their pros and cons in the commercial production of Halal meat.

4.
Food Sci Nutr ; 9(8): 4048-4056, 2021 Aug.
Article in English | MEDLINE | ID: mdl-34401056

ABSTRACT

Pulses have been an excellence source of foods due to their nutritional profile including high protein content. In addition, they have less concerns about allergens, gluten, and genetically modified organisms. In this study, high moisture meat analogs (HMMA) that contained commercial pea protein (55.4% protein), lentil protein (55.4% protein), or faba bean protein (61.5% protein) mixed with other constant ingredients (pea isolates, and wheat gluten and canola oil) were produced using a twin-screw extruder (TX-52) with an attached cooling chamber. For consumer sensory tests and texture profile analysis, vegetable hamburger patties with HMMA were produced with the addition of spices, binders, and so on. Trained panelists reported that HMMA with pulses had higher scores on bean-like, sweet, and cohesiveness of mass compared to HMMA with soy that had higher soy, cardboardy, hardness, and springiness scores. Compared to the control, consumer panelists indicated that samples containing pulse proteins had no differences in consumers' liking for the cooked appearance and overall flavor, but had a lower overall texture liking score, and samples with faba bean proteins (FP) had a lower overall liking score. Cooked patties containing pulse proteins were redder and had more cooking yield. Patties with FP required less cooking time. Therefore, vegetable patties with pulse proteins are competitive with soy-based samples.

5.
J Food Sci ; 86(4): 1322-1334, 2021 Apr.
Article in English | MEDLINE | ID: mdl-33761139

ABSTRACT

Pulse proteins (PLP) can be ideal alternative-sources that produce a meat-like textured product, known as a high moisture meat analog (HMMA). In this research, each commercial PLP: pea (16%), lentil (16%), and faba-bean (20%) was mixed with pea isolate (63%, 63%, and 59%, respectively) and constant ingredients which are canola oil (6%) and wheat gluten (15%) and texturized to produce HMMA using a twin-screw extruder (TX-52) with a cooling die. Soy concentrate and soy isolate were mixed with the constant ingredients and texturized into an HMMA and used as a control. Before freezing for storage, each sample was cooled by air, water, or a brine solution (2% or 4%) for 10 min. Frozen samples were thawed at room temperature (25 °C) for 3 hr and rehydrated by soaking at 25 °C for 2 hr, warm-soaking at 50 °C for 12 hr, or boiling for 2 min. Color, moisture content (MC), specific density (SD), water absorption index (WAI), water solubility index (WSI), and texture were measured. Compared to the control, samples with PLP had less lightness and texture and greater redness, yellowness, MC and WSI. The 2% brine solution used for cooling reduced WSI without textural change compared to other cooling methods. Boiling for rehydration increased lightness while warm-soaking decreased lightness and increased yellowness. In addition, boiling resulted in the least MC, SD, WSI, and WAI following soaking and warm-soaking. Therefore, these PLP can be used as alternative meat sources to soy proteins and a 2% brine solution for cooling and rehydration by boiling are recommended to reduce the WSI. PRACTICAL APPLICATION: Pulses are an excellent food ingredient because they are rich in protein and have an exceptional nutritional profile. In this study, high moisture meat analogs containing pea proteins, lentil proteins, faba bean proteins, and pea isolate instead of soy concentrate and soy isolate were produced. According to the results, pulse proteins can be an alternative source to soy proteins. Since they formed relatively well-defined orientation. Further research can be conducted using modified processing conditions for texturization to improve its quality. In addition, this research can help researchers and product developers understand proper handling methods for HMMA products after production such as cooling before freezing for storage and thawing and rehydrating after freezing.


Subject(s)
Cold Temperature , Fluid Therapy , Food, Formulated/analysis , Lens Plant/chemistry , Meat , Pea Proteins/chemistry , Pisum sativum/chemistry , Vicia faba/chemistry , Water/chemistry
6.
Article in English | AIM (Africa) | ID: biblio-1264491

ABSTRACT

In Cote d'Ivoire, the prevalence of HIV/AIDS has declined significantly in the last decade as a result ofpublic health actions. An assessment of knowledge, attitudes, perception and behaviours was carriedout to assess the outcome of the strategies for raising awareness and communicating with students about HIV/AIDS. A descriptive cross-sectional study was conducted in 2014 among students enrolled in third year at the university. An anonymous self-questionnaire including a consent form was provided. Mean scores were calculated from the items in the questionnaire to assess the level of knowledge, attitude, perception and behaviours of these students with regard to HIV/AIDS. The chi square test was used to compare the qualitative variables, and the quantitative variables were compared with the test of student. Among 561 students who participated in the study, 41.7, 26.7 and 31.6% were respectively registered in the sectors of human and social sciences (HSS), biosciences and health sciences. The mean age was 25.9 ± 3.4 years with a sex ratio of 1.3. The mean knowledge score of certain modes of transmission was 4.9 ± 0.36 out of 5 while that of condom positive perception was 6.4 ± 2.2 out of 12. This perception score was significantly higher in women than in men (6.1 vs. 6.9, p = 0.0001). The mean acceptance score was 7.3 ± 2.2 out of 10. The results of this work show good knowledge about HIV/AIDS among the students surveyed. However, the positive perception of the condom is far from being integrated in the student environment. This study also showed the persistence of HIV risk behaviour is out of sync with the level of Knowledge. It would be important to review marketing and communication strategies about HIV


Subject(s)
Acquired Immunodeficiency Syndrome/psychology , Behavior , Cote d'Ivoire , Cross-Sectional Studies , HIV Infections/epidemiology , Health Knowledge, Attitudes, Practice , Students
7.
Front Microbiol ; 9: 1200, 2018.
Article in English | MEDLINE | ID: mdl-29922269

ABSTRACT

Due to the high similarity in their requirements for space and food, close bacterial relatives may be each other's strongest competitors. Close bacterial relatives often form visible boundaries to separate their swarming colonies, a phenomenon termed colony-merger incompatibility. While bacterial species are known to have many incompatible strains, it is largely unclear which traits lead to multiple incompatibilities and the interactions between multiple incompatible siblings. To investigate the competitive interactions of closely related incompatible strains, we mutated Myxococcus xanthus DK1622, a predatory bacterium with complex social behavior. From 3392 random transposon mutations, we obtained 11 self-identification (SI) deficient mutants that formed unmerged colony boundaries with the ancestral strain. The mutations were at nine loci with unknown functions and formed nine independent SI mutants. Compared with their ancestral strain, most of the SI mutants showed reduced growth, swarming and development abilities, but some remained unchanged from their monocultures. When pairwise mixed with their ancestral strain for co-cultivation, these mutants exhibited improved, reduced or unchanged competitive abilities compared with the ancestral strain. The sporulation efficiencies were affected by the DK1622 partner, ranging from almost complete inhibition to 360% stimulation. The differences in competitive growth between the SI mutants and DK1622 were highly correlated with the differences in their sporulation efficiencies. However, the competitive efficiencies of the mutants in mixture were inconsistent with their growth or sporulation abilities in monocultures. We propose that the colony-merger incompatibility in M. xanthus is associated with multiple independent genetic loci, and the incompatible strains hold competitive interaction abilities, which probably determine the complex relationships between multiple incompatible M. xanthus strains and their co-existence strategies.

8.
Crit Rev Food Sci Nutr ; 53(11): 1168-79, 2013.
Article in English | MEDLINE | ID: mdl-24007421

ABSTRACT

Pulses are highly nutritious seeds of pod-bearing leguminous plants, specifically dry peas, lentils, and chickpeas. US farmers harvest about 2.6 million pounds of pulses every year but 75% of this is being exported internationally because of its increased consumption in the developing countries. In the current scenario, increasing costs of production, bad economy, and fluctuating food commodity prices have made a strong case for US producers to seek opportunities to increase domestic consumption of pulses through value-added products. Pulses are the richest sources of plant proteins and provide approximately 10% of the total dietary requirements of the proteins world over. Pulses are also high in dietary fibers and complex carbohydrates leading to low GI (glycemic index) foods. Pulses help to lower cholesterol and triglycerides as leguminous fibers are hypoglycosuria because of consisting more amylose than amylopectin. Pulses provide tremendous opportunities to be utilized in the processed foods such as bakery products, bread, pasta, snack foods, soups, cereal bar filing, tortillas, meat, etc. These show excellent opportunities in frozen dough foods either as added flour or as fillings. Pulses in view of their nutrient profile, seem to be ideal for inclusion in designing snack foods, baby, and sports foods.


Subject(s)
Diet , Fabaceae , Nutritive Value , Cajanus/chemistry , Cicer/chemistry , Dietary Carbohydrates/analysis , Dietary Fiber/analysis , Dietary Proteins/analysis , Fabaceae/chemistry , Food Handling , Glycemic Index , Glycosuria/prevention & control , Health Promotion , Humans , Hypolipidemic Agents , Lens Plant/chemistry , Pisum sativum/chemistry , Seeds , United States
9.
Clin Genet ; 78(5): 411-7, 2010 Nov.
Article in English | MEDLINE | ID: mdl-20653694

ABSTRACT

It is not known to what extent participation in a genetic testing program for BRCA1 and BRCA2, which does not include an extensive pre-test counselling session, influences cancer-related distress, cancer risk perception and patient satisfaction. Unselected Jewish women in Ontario were offered genetic testing for three common Jewish BRCA mutations. Before testing and 1-year post-testing, the women completed questionnaires which assessed cancer-related distress, cancer risk perception, and satisfaction. A total of 2080 women enrolled in the study; of these, 1516 (73%) completed a 1-year follow-up questionnaire. In women with a BRCA mutation, the mean breast cancer risk perception increased from 41.1% to 59.6% after receiving a positive genetic test result (p = 0.002). Among non-carriers, breast cancer risk perception decreased slightly, from 35.8% to 33.5% (p = 0.08). The mean level of cancer-related distress increased significantly for women with a BRCA mutation, but did not change in women without a mutation; 92.8% expressed satisfaction with the testing process. The results of this study suggest that the majority of Jewish women who took part in population genetic screening for BRCA1 and BRCA2 were satisfied with the delivery of genetic testing and would recommend testing to other Jewish women. However, women with a BRCA mutation experienced increased levels of cancer-related distress.


Subject(s)
Breast Neoplasms/genetics , Genes, BRCA1 , Genes, BRCA2 , Jews/genetics , Ovarian Neoplasms/genetics , Patient Satisfaction , Adult , Aged , Female , Genetic Carrier Screening , Genetic Counseling , Genetic Testing , Humans , Middle Aged , Ontario , Risk Factors
10.
Crit Rev Food Sci Nutr ; 49(4): 361-8, 2009 Apr.
Article in English | MEDLINE | ID: mdl-19234945

ABSTRACT

Vitamins (fat and water soluble) are vital food ingredients for healthy living, required by our bodies for normal metabolism. These are present in most natural food in small quantities, but when we process food through thermal processing methods (especially extrusion) a reasonable amount of the present vitamins are lost. During extrusion, factors like barrel temperature, screw rpm, moisture of ingredients, die diameter, and throughput affect the retention of vitamins in food and feed. The vitamins most sensitive to the extrusion process are vitamin A and vitamin E from fat-soluble vitamins, and vitamin C, B(1), and folic acid from water-soluble vitamins. The other vitamins of the B group, such as B(2), B(6), B(12), niacin, Ca-pantothenate, and biotin, are stable. Vitamin E itself or in its complex form is quite unstable during processing and even in storage of extruded food. Ascorbic acid directly added or coated with fat and then added to feed during extrusion is also very unstable. Vitamins A, C, D, and E are also sensitive to oxidation, so these vitamins have minimum retention during storage of extruded food.


Subject(s)
Food Handling/methods , Vitamins/chemistry , Drug Stability , Fats , Hot Temperature , Pressure , Solubility , Vitamin A/chemistry , Vitamin E/chemistry , Water
11.
J Inherit Metab Dis ; 26(2-3): 119-34, 2003.
Article in English | MEDLINE | ID: mdl-12889655

ABSTRACT

The majority of secreted or membrane-bound proteins are glycosylated. The glycans attached to glycoproteins can affect a range of physicochemical and biological properties of the glycoprotein and appropriate glycosylation is essential for many normal cellular functions, with aberrant glycosylation often leading to disease. This short review briefly outlines the methodology used to release glycans from proteins and analyse them by mass spectrometry. The technology is illustrated by the description of a rapid and sensitive method for profiling glycoproteins of patients with congenital disorders of glycosylation type II. This methodology can rapidly pinpoint the defective step(s) in the processing pathway of N-linked glycans, thereby focusing the biochemical analyses that need to be performed to define the genetic basis of these diseases.


Subject(s)
Congenital Disorders of Glycosylation/etiology , Glycoproteins/chemistry , Polysaccharides/chemistry , Congenital Disorders of Glycosylation/metabolism , Humans , Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization
13.
J Inherit Metab Dis ; 23(2): 162-74, 2000 Mar.
Article in English | MEDLINE | ID: mdl-10801058

ABSTRACT

18 UK patients (14 families) have been diagnosed with the carbohydrate-deficient glycoprotein syndrome (CDGS), type 1, on the basis of their clinical symptoms and/or abnormal electrophoretic patterns of serum transferrin. Eleven out of the 16 infants died before the age of 2 years. Patients from 12 families had a typical type 1 transferrin profile but one had a variant profile and another, who had many of the clinical features of CDGS type 1, had a normal profile. Eleven of the patients (10 families) with the typical type 1 profile had a deficiency of phosphomannomutase (PMM), (CDGS type 1a) but there was no correlation between residual enzyme activity and severity of disease. All these patients were compound heterozygotes for mutations in the phosphomannomutase (PMM2) gene, with 7 out of the 10 families having the common R141H mutation. Eight different mutations were found, including three novel ones. There was no correlation between genotype and phenotype, although siblings had similar phenotypes. Three patients, including the one with the normal transferrin profile, did not have a deficiency of phosphomannomutase or phosphomannose isomerase (CDGS 1b).


Subject(s)
Congenital Disorders of Glycosylation/genetics , Cells, Cultured , Congenital Disorders of Glycosylation/physiopathology , Congenital Disorders of Glycosylation/psychology , DNA Mutational Analysis , Female , Fibroblasts/enzymology , Genotype , Humans , Infant , Infant, Newborn , Lymphocytes/enzymology , Male , Phenotype , Reverse Transcriptase Polymerase Chain Reaction , Transferrin/genetics , Transferrin/metabolism
14.
J Rheumatol ; 26(1): 78-80, 1999 Jan.
Article in English | MEDLINE | ID: mdl-9918244

ABSTRACT

OBJECTIVE: In view of evidence that stromelysin-1 and collagenase-1 are involved in tissue injury in inflammatory joint diseases, we sought to determine whether matrix metalloproteinases (MMP) are implicated in the pathophysiology of systemic lupus erythematosus (SLE). METHODS: Seventy-three patients with SLE and 39 healthy subjects were evaluated. Serum levels of MMP and tissue inhibitor of metalloproteinases were measured. RESULTS: Serum stromelysin-1 levels were significantly increased in patients with SLE (416+/-252 ng/ml) compared to healthy subjects (125+/-93 ng/ml). No correlation between serial measurements of stromelysin-1 and disease activity in SLE patients was noted. Serum collagenase-1, gelatinase A, and TIMP-1 levels were not increased in SLE. CONCLUSION: Serum concentrations of stromelysin-1 are increased in SLE, but the levels do not correlate with disease activity.


Subject(s)
Lupus Erythematosus, Systemic/blood , Matrix Metalloproteinase 3/blood , Disease Progression , Humans , Immunohistochemistry , Lupus Erythematosus, Systemic/enzymology , Lupus Erythematosus, Systemic/physiopathology , Metalloendopeptidases/blood , Prospective Studies , Tissue Inhibitor of Metalloproteinase-1/blood
15.
Prenat Diagn ; 18(7): 693-9, 1998 Jul.
Article in English | MEDLINE | ID: mdl-9706650

ABSTRACT

Two pregnancies at risk for the carbohydrate-deficient glycoprotein syndrome Type 1A (CDG1A, phosphomannomutase deficient) were monitored by enzyme and genetic linkage analyses. The index case in both families had a proven deficiency of phosphomannomutase (PMM). An unaffected fetus was predicted in family 1 following amniocentesis. Normal PMM activity was found in cultured amniotic fluid cells and there was no elevation of lysosomal enzymes in the amniotic fluid. Genetic linkage analysis using microsatellite markers closely linked to the CDG1A gene confirmed this prediction. A healthy child was born. In the second family direct assay of chorionic villi showed a profound deficiency of PMM and genetic linkage analysis showed the fetus to have the same haplotype as the proband. The pregnancy was terminated and a deficiency of PMM was confirmed in cultured fibroblasts from the fetus. Reliable prenatal diagnosis of CDG Type 1A (PMM-deficient) can be achieved by a combination of biochemical and molecular genetic tests.


Subject(s)
Amniocentesis , Chorionic Villi Sampling , Congenital Disorders of Glycosylation/diagnosis , Genetic Linkage , Phosphotransferases (Phosphomutases)/deficiency , Amniotic Fluid/cytology , Amniotic Fluid/enzymology , Cells, Cultured , Female , Fibroblasts/enzymology , Humans , Lysosomes/enzymology , Male , Microsatellite Repeats , Pedigree , Phosphotransferases (Phosphomutases)/analysis , Pregnancy
16.
Glycobiology ; 8(4): 351-7, 1998 Apr.
Article in English | MEDLINE | ID: mdl-9499382

ABSTRACT

The glycosylation of serum transferrin from galactosemic patients with a deficiency of galactose-1-phosphate uridyl transferase (EC 2. 7.7 12) is abnormal but becomes normal after treatment with a galactose-free diet. To understand the structural and biochemical basis of the abnormal glycosylation, transferrin was purified from the serum of untreated and treated galactosemic patients and normal controls and the N-linked glycans analyzed by HPLC. The glycans from normal transferrin consisted predominantly (86%) of the disialylated biantennary complex type. The glycans from untreated galactosemic patients were more heterogeneous and contained four major truncated glycans in addition to a smaller amount (13%) of the disialylated biantennary complex type. The truncated glycans were deficient in galactose and sialic acid and their structures were consistent with a decrease in galactosyltransferase activity in hepatocytes, the probable cells of origin of the transferrin. This is postulated to be due to direct inhibition of the galactosyltransferase activity by the accumulated galactose-1-phosphate or to an effect on the formation of UDP-galactose, the donor substrate in the reaction. After treatment the proportion of the truncated glycans decreased and the proportion of the disialylated biantennary complex type increased, returning almost but never completely to normal, even after prolonged treatment in some cases. There was no clear relationship between the length of treatment and the normalization of glycosylation and the level of galactose-1-phosphate in red blood cells, the usual parameter for monitoring the treatment of galactosemics. It is suggested that the persistence of abnormally glycosylated proteins may contribute to the long-term complications in galactosemia.


Subject(s)
Galactosemias/blood , Transferrin/chemistry , Carbohydrate Sequence , Chromatography, High Pressure Liquid , Dietary Carbohydrates/administration & dosage , Erythrocytes/metabolism , Galactose/administration & dosage , Galactose/chemistry , Galactosemias/diet therapy , Galactosephosphates/blood , Glycosylation , Humans , Molecular Sequence Data , Molecular Structure , Polysaccharides/chemistry , UDPglucose-Hexose-1-Phosphate Uridylyltransferase/deficiency
17.
Acad Emerg Med ; 4(12): 1118-21, 1997 Dec.
Article in English | MEDLINE | ID: mdl-9408426

ABSTRACT

OBJECTIVE: To determine in adult medical patients the incidence of deep venous thrombosis (DVT) resulting from femoral venous catheterization (FVC). METHODS: A prospective, observational study was performed at a 420-bed community teaching hospital. Heparin-coated 7-FR cm femoral venous catheters were inserted unilaterally into a femoral vein. Each contralateral leg served as a control site. Age, gender, number of FVC days, DVT risk factors, administration of DVT prophylaxis, and DVT formation and site were tabulated for each patient. Venous duplex sonography was performed bilaterally on each patient within 7 days of femoral venous catheter removal. RESULTS: Catheters were placed in 29 men and 13 women. Femoral DVT was identified by venous duplex sonography in 11 (26.2%) of the FVC legs and none (0%) in the control legs. Posterior tibial and popliteal DVT was identified in both the FVC and control legs of 1 patient. DVT formation at the site of FVC insertion was highly significant (p = 0.005). There were no statistically significant associations with age (p = 0.42), gender (p = 0.73), number of DVT risk factors (p = 0.17), number of FVC days (p = 0.89), or DVT prophylaxis (p = 0.99). CONCLUSION: Placement of femoral catheters for central venous access is associated with a significant incidence of femoral DVT as detected by venous duplex sonography criteria at the site of femoral venous catheter placement. Physicians must be aware of this risk when choosing this vascular access route for adult medical patients. Further studies to assess the relative risk for DVT anf its clinical sequelae when using the femoral vs other central venous catheter routes are indicated.


Subject(s)
Catheterization, Peripheral/adverse effects , Femoral Vein , Thrombosis/etiology , Adult , Aged , Female , Humans , Incidence , Male , Middle Aged , Prospective Studies , Risk Factors , Thrombosis/diagnostic imaging , Ultrasonography, Doppler, Duplex
18.
J Inherit Metab Dis ; 20(6): 817-26, 1997 Nov.
Article in English | MEDLINE | ID: mdl-9427152

ABSTRACT

The carbohydrate-deficient glycoprotein syndrome (CDGS) is a group of disorders characterized biochemically by abnormal glycosylation of serum and cellular glycoproteins. It has been classified into four forms on the basis of the isoelectric focusing pattern of serum transferrin and difference in clinical presentation. A deficiency of phosphomannomutase (PMM) has been reported in most patients with type 1. Seven of our eight CDGS patients, classified clinically as type 1, were shown to have a deficiency of phosphomannomutase in their fibroblast or lymphoblastoid cells (0.04-0.2 nmol/min per mg, compared with a control range of 1.0-2.1 nmol/min per mg). The eighth patient, who had many clinical features of the severe neonatal form of CDGS type 1, but lacked definite signs of CNS and ocular involvement, had a normal phosphomannomutase activity in his fibroblasts. There were approximately equal amounts of disialo- and tetrasialotransferrin and only a trace amount of asialotransferrin in the serum and ascitic fluid of this patient. The disialo- and tetrasialotransferrin isoforms were purified by ion-exchange chromatography and analysed by SDS-PAGE. The disialotransferrin had a lower molecular mass than the tetrasialotransferrin, consistent with the absence of an N-linked glycan. The N-linked glycans released enzymically from both isoforms consisted exclusively of disialylated biantennary chains, suggesting that disialotransferrin results from underglycosylation, as in the PMM-deficient CDGS type 1 patients. It is concluded that the clinical and biochemical phenotype in CDGS type 1 can result from more than one basic defect.


Subject(s)
Congenital Disorders of Glycosylation/diagnosis , Phosphotransferases (Phosphomutases)/metabolism , Ascitic Fluid/chemistry , Carbohydrate Conformation , Chromatography, Ion Exchange , Congenital Disorders of Glycosylation/enzymology , Electrophoresis, Polyacrylamide Gel , Fibroblasts/enzymology , Glycosylation , Humans , Infant, Newborn , Isoelectric Focusing , Male , Polysaccharides/analysis , Transferrin/analysis
19.
Glycoconj J ; 13(6): 1031-42, 1996 Dec.
Article in English | MEDLINE | ID: mdl-8981095

ABSTRACT

One of the biochemical characteristics of carbohydrate deficient glycoprotein syndromes is the presence of abnormal glycoforms in serum transferrin. Both glycoform heterogeneity and variable site occupancy may, in principle, lead to the generation of a range of glycoforms which contain different numbers of sialic acid residues, and therefore variable amounts of negative charge. Capillary zone electrophoresis was used to resolve the glycoforms of normal human serum transferrin and also of a set of glycoforms which were prepared by digesting the sugars on the intact glycoprotein with sialidase. The sugars on the intact glycoprotein were also modified by a series of exoglycosidase enzymes to produce a series of neutral glycoforms which were-also analysed by capillary zone electrophoresis. The oligosaccharide population of human serum transferrin was analysed by a series of mixed exoglycosidase digests on the released glycan pool and quantified using a novel HPLC strategy. Transferrin was isolated from carbohydrate deficient glycoprotein syndromes type I serum and both the intact glycoforms and released sugars were resolved and quantified. The data presented here confirm the presence of a hexa-, penta- and tetra-sialoforms of human serum transferrin in both normal and carbohydrate deficient glycoprotein syndrome type I serum samples. Consistent with previous reports carbohydrate deficient glycoprotein syndrome type I transferrin also contained a di-sialoform, representing a glycoform in which one of the two N-glycosylation sites is unoccupied, and a non-glycosylated form where both remain unoccupied. This study demonstrates that capillary zone electrophoresis can be used to resolve quantitatively both sialylated and neutral complex type glycoforms, suggesting a rapid diagnostic test for the carbohydrate deficient glycoprotein syndromes group of diseases.


Subject(s)
Congenital Disorders of Glycosylation/metabolism , Electrophoresis, Capillary/methods , Transferrin/analysis , Transferrin/chemistry , Carbohydrates/analysis , Carbohydrates/chemistry , Chromatography, High Pressure Liquid , Congenital Disorders of Glycosylation/blood , Congenital Disorders of Glycosylation/diagnosis , Glycoside Hydrolases/metabolism , Humans , N-Acetylneuraminic Acid/analysis , Neuraminidase/metabolism , Polysaccharides/analysis , Polysaccharides/blood , Polysaccharides/chemistry , Sensitivity and Specificity , Transferrin/metabolism , beta-Galactosidase/metabolism
SELECTION OF CITATIONS
SEARCH DETAIL