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Results 401 - 450 of 760
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Vieira-Makings E - - 1991
Cobalamin neuropathy was produced in Cape fruit bats (Rousettus aegyptiacus) by a cobalamin-free diet combined with intermittent exposure to nitrous oxide, which inactivates cobalamin. Cobalamin-deficient bats had low hepatic methylmalonyl-CoA mutase holoenzyme activity, with elevated plasma and urinary methylmalonic acid levels. No significant changes could be demonstrated in the concentration ...
Davidson B L - - 1991
Complete hypoxanthine-guanine phosphoribosyltransferase (HPRT) deficiency causes the Lesch-Nyhan syndrome, an X-linked, purine metabolism disorder manifested by hyperuricemia, hyperuricaciduria, and neurologic dysfunction. Partial HPRT deficiency causes hyperuricemia and gout. One requirement for understanding the molecular basis of HPRT deficiency is the determination of which amino acids in this salvage enzyme are ...
Montgomery J A - - 1991
Riboflavin-deficient rats are used to study the metabolism of deuterium-labeled nonanoic acids under conditions mimicking the human disorder of multiple acyl-CoA dehydrogenase deficiency in which large amounts of ethyl-malonic, glutaric, adipic, suberic, 4-octenedioic, sebacic and 4-decenedioic acids are excreted. Both control and deficient rats convert the nonanoic acids to labeled ...
Demaugre F - - 1991
Reported cases of carnitine palmitoyltransferase II (CPT II) deficiency are characterized only by a muscular symptomatology in young adults although the defect is expressed in extra-muscular tissues as well as in skeletal muscle. We describe here a CPT II deficiency associating hypoketotic hypoglycemia, high plasma creatine kinase level, heart beat ...
Duranthon V - - 1991
The effect of essential fatty acid (EFA) deficiency on the lipid composition of basolateral plasma membranes (BPM) from intestinal mucosal cells was investigated in weaning pigs fed control or EFA-deficient diets for 12 weeks. The phospholipid and cholesterol contents relative to protein were similar in both groups, showing a cholesterol/phospholipid ...
Debry G - - 1991
The 'essentiality' of (omega-6) and (omega-3) fatty acids in mammals is well known. Nevertheless, some important points remain unclear concerning their implication in physiology. After a short discussion about the definition of essential fatty acids deficiency, this brief overview deals with some of these points, pointing out some of the ...
Laorpatanaskul S - - 1991
Fifteen patients with chronic renal failure, stabilized on twice weekly hemodialysis, received oral essential amino acid therapy (6.3 g/day) over a period of 12.3 months. Clinical and laboratory improvement was observed with respect to body weight, tricep skin fold thickness, mid upper arm circumference serum albumin, C3 and plasma essential ...
Taylor R G - - 1991
Histidase (histidine ammonia-lyase, EC 4.3.1.3) catalyzes the deamination of L-histidine to trans-urocanic acid in the liver and skin of mammals. Histidase deficiency results in increased histidine and histamine in blood, and decreased urocanic acid in blood and skin. In this review we discuss current research on: (1) the mechanism of ...
Marín M C - - 1991
The influence of severe protein-calorie malnutrition on essential fatty acid (EFA) status was evaluated in nursing infants aged 2-5 mo. A control group of the same age and with normal weight-for-height was also selected for this study. The fatty acid pattern of total phospholipids from plasma and red blood cells ...
Lefkowith J B - - 1991
Dietary polyunsaturated fatty acid manipulation exerts a strikingly protective effect in models of tissue inflammation and injury. A critical element of this effect appears to revolve around leukocyte trafficking but underlying mechanisms are ill understood. In the current study it was observed that essential fatty acid (EFA) deficiency markedly impaired ...
Wong K S - - 1991
Late-onset acid maltase deficiency is a rare disorder. We describe a nineteen year old Chinese girl who presented with diarrhoea, limb-girdle weakness and respiratory failure requiring mechanical ventilation. Electromyography showed polyphasic potentials and myotonic discharges. Muscle biopsy revealed features characteristic of acid maltase deficiency. Assay of acid alpha-glucosidase in cultured ...
Roufs J B - - 1991
ALS is a fatal, neurological disease of unknown origin with no present cure. A recent pilot study has shown that L-threonine, an essential amino acid, may be effective in the symptomatic treatment of ALS. Relevant information discussing the potential role of L-threonine in the treatment of ALS is presented.
Honda M - - 1991
In young children on CAPD, hypoproteinemia and malnutrition are often observed. We used essential amino acid-containing dialysate (EAAD) to assess short-term effectiveness on serum amino acid concentrations in young children undergoing CAPD. EAAD consisted of a 540 ml, 1.5% glucose-containing dialysate and 100 ml of 7.4% essential amino acid (EAA) ...
Koletzko B - - 1991
Growth failure is a classical sign of essential fatty acid deficiency. We investigated whether birth weight correlates with the postnatal essential fatty acid status in a group of 29 premature infants. A significant and positive correlation between body weight and plasma triglyceride content of arachidonic acid (20:4n-6) (r = 0.47, ...
Porteres G A - - 1991
1. The fatty acid composition of short-neck clams (Ruditapes philippinarum A & R) was compared in animals (a) fed on industrial yeast, (b) fed on natural phytoplankton, (c) after starvation. 2. Marked deficiencies in polyunsaturated fatty acids were found in the case of a yeast diet as well as after ...
Crawford M A - - 1991
Evidence that changes in feeding style alter the membrane fatty acid composition of ruminant tissue is presented here by comparing zoo giraffe with the same species from their natural habitat. The membrane changes seen are similar to those used experimentally to make animals susceptible to basic brain protein and encephalomalacia. ...
Nilsson A - - 1990
Lipoprotein lipase (LPL) activities of postheparin plasma, heart, lungs, and adipose tissue, and salt-resistant lipase (hepatic lipase, SRL) activities of postheparin plasma, liver, and adrenals were examined in essential fatty acid deficient (EFAD) rats and in age-matched controls. The LPL activity of heart was higher in the deficient than in ...
Lehmann A - - 1990
In taurine-deficient cats, the secretion of bile acids is impaired, and this impairment may reduce intestinal uptake of lipophilic vitamins. It was therefore hypothesized that retinol deficiency is involved in the generation of retinal lesions in taurine-deficient kittens. To this end, the concentration of retinol in plasma and liver was ...
Hjelte L - - 1990
1. Renal function was studied in 50-, 70-, 90- and 200-day-old rats with essential fatty acid deficiency. The pharmacokinetics of tobramycin was investigated in 90-day-old essential fatty acid-deficient rats. 2. A higher glomerular filtration rate and a higher serum concentration of urea were seen in 50-day-old essential fatty acid-deficient rats ...
Valentich M A - - 1990
The effect of essential fatty acid deficiency and nitrosomethylurea treatment on postnatal levels of pancreatic gamma-glutamyltranspeptidase was studied. A significant increase of gamma-glutamyltranspeptidase activity and changes in fatty acid composition were observed in essential fatty acid deficient rats, from the 14th day of life on. Pancreatic gamma-glutamyltranspeptidase of nitrosomethylurea injected ...
Honda M - - 1990
Studies were performed on the transfer of essential and non-essential non-esterified fatty acids across the in-situ perfused placenta in normal and diabetic rats in late gestation. Results indicated that increasing maternal glycaemia is associated with a decrease in the unidirectional transfer of both essential and non-essential fatty acids. This reduced ...
Léger C L - - 1990
ESR spectrometry with 5-, 7-, 10-, and 12-doxylstearate probes and a combined index considering separately the double-bond numbers of essential and nonessential fatty acids were used to investigate the structural role of the double bonds of polyunsaturated fatty esters in membrane phosphoglycerides. Purified brush border membrane vesicles were prepared from ...
Jamal G A - - 1990
A substantial disturbance of the metabolism of the n-6 essential fatty acids exists in both human and experimental diabetes mellitus. Disturbances of the essential fatty acids and of the 1- and 2-series prostaglandins derived from them create a variety of microvascular, haemorheological, and other abnormalities leading to reduced blood flow ...
Cunnane S C - - 1990
The rat mesenteric vascular bed releases prostaglandins when perfused in vitro. The present study evaluated the effect of perfusion of the rat mesenteric vascular bed in vitro with a buffer containing 0, 3, 6, or 9 nM of added zinc on the release of essential fatty acids over a 150-min ...
Sattar A - - 1990
Influence of irradiation (0.05-0.20 kGy) and germination (24-120 hours) in distilled and tap water on phytate, protein and amino acids of soybean, was studied. Phytate values significantly decreased with increasing germination period and irradiation dose (P less than 0.01). Irradiation independently decreased the original phytate (212.0 mg/100 g) to a ...
Takeuchi M - - 1990
3-Ketovalidoxylamine A C-N lyase of Flavobacterium saccharophilum is a monomeric protein with a molecular weight of 36000, and contains 32 amino groups and no cysteine or cystine residues. The enzyme was inactivated by 2,4,6-trinitrobenzenesulfonic acid (TNBS) following pseudo-first order kinetics. Substrate of the lyase, p-nitrophenyl-3-ketovalidamine, protected the enzyme against the ...
Grattan C - - 1990
The concentration of essential fatty acids (EFAs) and their metabolites in plasma phospholipids were measured by gas chromatography in normal individuals, and in patients with ichthyosis vulgaris, acne vulgaris or psoriasis. In all three patient groups, concentrations of arachidonic acid (20:4 omega 6) and docosapentaenoic acid (22:5 omega 6) were ...
Proud V K - - 1990
Full-thickness biopsies of haired and alopecic skin of biotin-deficient rats had less subcutaneous fat and showed lipophilic follicular plugging, vascular engorgement, epidermal hyperplasia, and abnormal keratinization. Mean activities of the three mitochondrial biotin-dependent carboxylases in the skin of biotin-deficient animals were reduced to 3-18% of control whereas the cytosolic enzyme, ...
Alessandri J M - - 1990
The lipid components of columnar cells harvested from rat small intestine were analyzed at each step of cell maturation. The effect of dietary lipids on the evolution of lipids in differentiating cells was studied using two diets representative either of a control or of an essential polyunsaturated fatty acid deficient ...
Shimizu T - - 1990
The relationship between essential fatty acid (EFA) deficiency and disturbance of prostaglandin (PG) biosynthesis was studied in children after radical operation for extrahepatic biliary atresia (EBA). In addition, to investigate the method for treatment of postoperative EFA deficiency and disturbance of PG biosynthesis, the serum fatty acid and plasma PG ...
Levy J - - 1990
Essential fatty acid deficiency was documented in a 3-year-old boy with chronic cholestasis secondary to paucity of intrahepatic bile ducts (Alagille's syndrome). Dietary management had consisted almost exclusively of a proprietary formula with over 80% of the fat as medium-chain triglycerides. The bullous lesions involved mostly sun-exposed areas and were ...
Mock D M - - 1990
Abnormalities in fatty acid composition have been detected in biotin deficiency in humans and in the rat. However, the pathogenetic role of these abnormalities, if any, in the cutaneous manifestations of biotin deficiency is not clear. In a nutrient interaction experiment, we tested the hypothesis that an abnormality of omega ...
Reisbick S - - 1990
Omega-3 fatty acids are a major component of neural membranes. They are essential nutrients for normal biochemical development of the brain and retina and may affect behavior. In our studies of long-term dietary omega-3 fatty acid deficiency, we have found a new effect of this deficiency in rhesus monkeys. Deficient ...
Packman S - - 1990
Holocarboxylase synthetase (HS) and biotinidase deficiencies have been identified as causes of biotin-responsive multiple carboxylase deficiency. Acetyl-CoA carboxylase (ACC) deficiency has been shown to occur in multiple carboxylase deficiency, and HS(-) fibroblasts are being employed to investigate compensatory regulatory responses in cells deficient in ACC. In previous studies, biotin starved ...
Lefkowith J B - - 1990
EFA deficiency appears to be a uniquely useful tool in probing the role of arachidonate in a variety of physiologic and pathologic processes. Recent studies have clarified the effects of the deficiency state on tissue fatty acid content and on the production of metabolites of arachidonate and related mediators such ...
Dasgupta A - - 1990
Plasma fatty acid profiles from maintenance hemodialysis patients (n = 9) were compared with those from healthy volunteers (n = 9). Hemodialysis patients had significantly higher levels of oleic acid, 15.3 +/- 1.1 vs. 8.9 +/- 0.6% (p less than 0.0001), and lower levels of arachidonic acid (6.0 +/- 0.5 ...
Davidson B C - - 1990
Considerable evidence shows dietary nutrient variation of human foods, but little data is available for wild animals. Plants are known to be low in total lipid, thus limiting essential fatty acid availability to herbivores. The fatty acid profiles of a range of indigenous Southern African plants were examined. Significant differences ...
Schreiner G F - - 1989
Dietary polyunsaturated fatty acid modulation exerts a beneficial effect in immune-mediated glomerulonephritis. To elucidate the mechanisms underlying this phenomenon, the effects of essential fatty acid (EFA) deficiency on the heterologous phase of nephrotoxic nephritis in rats (induced by the injection of a rabbit antiglomerular basement membrane antibody) were studied. The ...
Rhodes D - - 1989
A hybrid of sweet corn, Zea mays L. (;1720'; Rogers Brothers Seed Co.), was found to be comprised of glycinebetaine-positive and glycinebetaine-deficient individuals in a 1:1 mixture. This phenomenon was traced to segregation for a single, nuclear, dominant gene determining leaf glycinebetaine content within the female inbred parent of this ...
Lepage G - - 1989
This study was aimed at redefining criteria for essential fatty acid (EFA) deficiency with the use of the direct transesterification procedure (1986. J. Lipid Res. 27: 114-120) and at determining whether a simple assay of total fatty acids (FA) is as predictive of EFA deficiency as the FA pattern from ...
Katz I R - - 1989
We have compared levels of albumin and serum amino acids in a group of 87 recent admissions to a nursing home, average age 83 years, with a group of healthy moderately old subjects, average age 69 years. We found that the nursing home group was characterized by decreased levels of ...
Maxton D G - - 1989
The presence of food within the small intestinal lumen promotes mucosal cell proliferation. To define the trophic role of triglycerides, three groups of eight female Wistar rats were isocalorically fed for four weeks with either Vivonex, or Vivonex with 50% calorie substitution with an essential fatty acid mixture, or Vivonex ...
Smith S S - - 1989
This study assessed the effect of a dietary deficiency in the essential fatty acids (EFA) linoleic and linolenic acids on the onset of female puberty. EFA deficiency was produced in female rats by means of a semipurified diet and was biochemically documented by analyzing serum and erythrocyte fatty acid levels ...
Strandvik B - - 1989
Changes in renal hemodynamics, sodium homeostasis, renal acidifying capacity, and aldosterone excretion were studied before and after long-term intravenous essential fatty acid supplementation for a period of 3 years in 11 patients with cystic fibrosis. The mean (+/- SD) glomerular filtration rate was high at the start of the study ...
Mahfouz M M - - 1989
Experimental Mg2+ deficiency was induced in a group of rats by feeding them a Mg2+-deficient diet for 23 days. They were pair-fed to compare with a control group of rats fed a Mg2+-sufficient diet. In the Mg2+-deficient group the plasma total cholesterol and triglyceride levels were increased while HDL-cholesterol was ...
Horrobin D F - - 1989
A deficiency of essential fatty acid intake can produce severe cutaneous abnormalities but is exceedingly rare in clinical practice. Recent research has shown that abnormalities in essential fatty acid metabolism may play a role in atopic eczema, acne, and psoriasis. Therapeutic innovations have already resulted from this knowledge, and more ...
Woody R C - - 1989
Hyperphenylalaninemia in infants and children may be caused by a deficiency of dihydropteridine reductase (DHPR). Recommended therapy includes folinic acid as a source of tetrahydrofolate, a phenylalanine-restricted diet, and both dopamine and serotonin precursors. We report a child with progressive basal ganglia and other subcortical calcifications prior to the use ...
Davidson B C - - 1989
The cat is deficient in delta-6-desaturase, preventing its utilisation of the plant-derived essential fatty acids. The species circumvents this problem by eating non-felines capable of this process. Kittens were fed diets varying in the type of post delta-6-desaturation fatty acids (PDFA). Growth patterns and external signs monitored for a period ...
Bull A W - - 1989
The essential fatty acid requirement for the development of intestinal carcinogenesis was determined and compared to the overall essential fatty acid status of the animals as measured by the triene/tetraene ratio in the plasma, liver and colon. To induce tumors, male Sprague-Dawley rats were given two weekly injections (20 mg/kg ...
Igarashi N - - 1989
We describe the fatty acid (FA) and prostaglandin (PG) metabolism in two Japanese cases of cystic fibrosis (CF) with or without pancreatic insufficiency (PI). The diagnosis of CF was based on the elevated sweat chloride concentration by pilocarpine iontophoresis. A 1-month-old boy (case 1) showed poor weight gain, steatorrhea and ...
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