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Yara Asanori - - 2008
Linolenic acid (18:3) and its derivative jasmonic acid (JA) are important molecules in disease resistance in many dicotyledonous plants. We have previously used 18:3- and JA-deficient rice (F78Ri) to investigate the roles of fatty acids and their derivatives in resistance to the blast fungus Magnaporthe grisea [A. Yara, T. Yaeno, ...
Luu Tony C TC Department of Pharmaceutics and Medicinal Chemistry, Thomas J. Long School of Pharmacy and Health Sciences, University of the Pacific, Stockton, CA 95211, - - 2008
Cyclophilin-40 (CyP40) promotes the formation of the gel shift complex that contains the aryl hydrocarbon receptor (AhR), AhR nuclear translocator (Arnt) and dioxin response element (DRE) using baculovirus expressed proteins. Here we reported that CyP40 plays a role in the AhR signaling. When the CyP40 content in MCF-7 cells is ...
Stoffel Wilhelm - - 2008
Mammalian cell viability is dependent on the supply of the essential fatty acids (EFAs) linoleic and alpha-linolenic acid. EFAs are converted into omega3- and omega6-polyunsaturated fatty acids (PUFAs), which are essential constituents of membrane phospholipids and precursors of eicosanoids, anandamide and docosanoids. Whether EFAs, PUFAs and eicosanoids are essential for ...
Taniguchi Ayumi - - 2008
Biotin is a water-soluble vitamin which functions as a coenzyme of carboxylases in glucose and amino acid metabolism and fatty acid synthesis. Biotin is also essential for maintaining reproductive function. Biotin deficiency during gestation induces cleft palate, micrognathia and limb hypoplasia in mouse fetuses at near term. Maternal biotin deficiency ...
McCullagh James - - 2008
This study provides data for the effect of dietary non-essential amino acid composition on the delta(13)C values of individual amino acids in rainbow trout (Oncorhynchus mykiss) using liquid chromatography coupled to isotope ratio mass spectrometry (LC/IRMS). In this experiment, trout were reared either on a control diet or on three ...
Schiaffino Stefano - - 2008
Autophagy is activated soon after birth in neonatal tissues and is essential for survival because mice deficient in Atg5 or Atg7 autophagy genes die within 1 day after birth. Amino acid starvation has been considered as a major deleterious effect of autophagy deficiency, since the concentration of amino acids in ...
- - 2008
(1) N-acetylglutamate synthase deficiency is a rare congenital disorder that causes hyperammonaemic comas, resulting in severe neurological morbidity and usually leading to death during childhood. (2) Carglumic acid is the first drug to be used for replacement therapy. Data available in 2003 showed beneficial effects on growth and psychomotor development. ...
Harel Moshe - - 2008
Recent evidence suggests that there is a dynamic microbial biota living on the surface and in the mucus layer of many hermatypic coral species that plays an essential role in coral well-being. Most of the studies published to date emphasize the importance of prokaryotic communities associated with the coral mucus ...
Essalmani Rachid - - 2008
The proprotein convertase PC5/6 cleaves protein precursors after basic amino acids and is essential for implantation in CD1/129/Sv/C57BL/6 mixed-background mice. Conditional inactivation of Pcsk5 in the epiblast but not in the extraembryonic tissue bypassed early embryonic lethality but resulted in death at birth. PC5/6-deficient embryos exhibited Gdf11-related phenotypes such as ...
Huang Chun Y - - 2008
Plants modify metabolic processes for adaptation to low phosphate (P) conditions. Whilst transcriptomic analyses show that P deficiency changes hundreds of genes related to various metabolic processes, there is limited information available for global metabolite changes of P-deficient plants, especially for cereals. As changes in metabolites are the ultimate 'readout' ...
Nowruzi Keyvan - - 2008
A systematic approach was developed to identify and optimize the essential amino acids in defined minimal medium for the production of recombinant human interleukin 3 (rHuIL-3) by Streptomyces lividans. Starvation trials were carried out initially to narrow down the number of probable essential amino acids from an initial number of ...
Kayrak Mehmet - - 2009
Essential thrombocythemia is a clonal myeloproliferative disorder that causes thrombocytosis. Essential thrombocythemia is characterized by increased incidence of thrombosis with arterial event more than venous events and hemorrhagic complications. Acetylsalicylic acid enhances both minor and major bleedings. The authors describe pericardial hemorrhage, which is related to the use of low-dose ...
Masood M Athar - - 2008
Fatty acid analysis is an important research tool, and indices derived from essential fatty acid contents serve as useful biomarkers related to cardiovascular and other chronic disease risk. Both clinical and basic studies of essential fatty acid composition are becoming ever larger in magnitude leading to delays while the rather ...
Delgado Carmen - - 2007
Propionic acidemia is a hereditary metabolic disease caused by a deficiency of enzyme propionyl-CoA carboxylase, which is involved in the catabolism of ramified amino acids, odd-chain fatty acids, and other metabolites; the deficiency of this enzyme leads to an accumulation of toxic substances in the body. There are various forms ...
Thompson S A - - 2008
Alpha-methylacyl-CoA racemase (AMACR) deficiency is a rare disorder of fatty acid metabolism which has recently been described in three adult cases. We have identified a further patient with clinical features of a relapsing encephalopathy, seizures and cognitive decline over a 40 year period. Biochemical studies revealed grossly elevated plasma pristanic ...
Wieneke Nadine - - 2007
A tight hormonal control of energy homeostasis is of pivotal relevance for animals. Recent evidence suggests an involvement of the nuclear receptor NR1i3 (CAR). Fasting induces CAR by largely unknown mechanisms and CAR-deficient mice are defective in fasting adaptation. In rat hepatocytes CAR was induced by WY14643, a PPARalpha-agonist. A ...
Rizzo William B - - 2008
Sjögren-Larsson syndrome (SLS) is an inherited neurocutaneous disorder characterized by ichthyosis, mental retardation, spasticity, and deficient activity of fatty aldehyde dehydrogenase (FALDH). FALDH is an enzyme component of fatty alcohol:NAD oxidoreductase (FAO), which is necessary for fatty alcohol metabolism. To better understand the biochemical basis for the cutaneous symptoms in ...
Behre Jörn - - 2008
We present a generalised framework for analysing structural robustness of metabolic networks, based on the concept of elementary flux modes (EFMs). Extending our earlier study on single knockouts [Wilhelm, T., Behre, J., Schuster, S., 2004. Analysis of structural robustness of metabolic networks. IEE Proc. Syst. Biol. 1(1), 114-120], we are ...
Hashimoto Kosuke - - 2008
The repertoire of biosynthetic enzymes found in an organism is an important clue for elucidating the chemical structural variations of various compounds. In the case of fatty acids, it is essential to examine key enzymes that are desaturases and elongases, whose combination determine the range of fatty acid structures. We ...
Kawasaki Shinji - - 2007
This report investigates the requirement for CO2 for colony formation by Bifidobacterium species in both anoxic and oxic environments. All tested Bifidobacterium species exhibited difficulty in developing colonies in an atmosphere of 100% N2 but developed well when 1% CO2 was present. In the presence of CO2, the oxygen tolerance ...
Yehuda Shlomo - - 2007
Rapid eye movements (REM) deprivation induces complex deteriorating effects, which include brain morphological changes such as reduced neurogenesis processes, brain neurochemical and hormonal modifications, and cognitive decline. One of the major effects of REM deprivation is an increased anxiety level. The aim of this study was to examine the effects ...
Dirik Eray - - 2008
A patient with isolated 3-methylcrotonyl-CoA carboxylase (MCC) deficiency with an unusual clinical presentation is described. The patient presented with clusters of seizures with two or three months disease free interval in the first year of life which then evolved into attacks of status epilepticus after the age of 12 months. ...
Bi J L - - 2007
The glassy-winged sharpshooter (GWSS) is an invasive insect pest in California that proliferated in citrus while incorporating many other plant species in its host range. Field studies were conducted from September 2002 to September 2003 in adjacent young and old orange groves to determine the influence of amino acid concentrations ...
Rodríguez-Fuentes Nayeli - - 2007
Pyruvate carboxylase (PC) is a biotin-dependent enzyme that plays a crucial role in gluconeogenesis, lipogenesis, Krebs cycle anaplerosis and amino acid catabolism. Biotin deficiency reduces its mass besides its activity. Enzyme mass is the result of its cellular turnover, i.e., its rates of synthesis and degradation. We have now investigated, ...
Teerawanichpan Prapapan - - 2007
Among the GFPs used for imaging green fluorescence, the Emerald version has been considered the best GFP to use but there is no formal report on its construction or the relevance of the amino acid (aa) substitutions in it relative to the commonly used GFPs. Here, we have shown that ...
Hyland Keith - - 2007
Many inherited disorders affecting aromatic amino acid metabolism have been described. This review will concentrate on the defects that lead to deficiencies of dopamine and serotonin within the central nervous system. Phenylalanine hydroxylase, tyrosine hydroxylase, and tryptophan hydroxylase all require tetrahydrobiopterin (BH4) as a cofactor. Inherited defects that reduce the ...
Dirkmann D - - 2007
Factor XI deficiency is a rare, hereditary bleeding disorder associated with a trauma-related bleeding tendency, caused by insufficient generation of the thrombin activatable fibrinolysis inhibitor (TAFI) evoking increased fibrinolysis. We present the case of a five year old girl with homozygote, severe factor XI deficiency presenting for surgery on two ...
Gomes Pedro - - 2007
In the yeast Saccharomyces cerevisiae, interventions resembling caloric restriction, either by reduction of glucose or non-essential amino acid content in the medium, prolong life span and retard aging. Here we have examined the role of auxotrophy-complementing amino acid supplementation of S. cerevisiae strains in determining yeast chronological life span and ...
Pearl Phillip L - - 2007
The pediatric neurotransmitter disorders represent an enlarging group of neurological syndromes characterized by abnormalities of neurotransmitter synthesis and breakdown. The disorders of dopamine and serotonin synthesis are aromatic amino acid decarboxylase deficiency, tyrosine hydroxylase deficiency, and disorders of tetrahydrobiopterin synthesis. Amino acid decarboxylase, tyrosine hydroxylase, sepiapterin reductase, and guanosine triphosphate ...
Dubnov-Raz Gal - - 2007
Several pregnant patients have asked recently whether media reports of children's intelligence quotients' increasing with increased intake of essential fatty acids are meaningful enough to be worth the effort of supplementation. What advice should they be given? Existing evidence from interventional studies is sparse and conflicting and should not lead ...
Matta Cecil A - - 2007
In the present report, we have determined the gonadal free amino acid compositions in Oreochromis niloticus collected from three sites in Lake Mariut: SE basin (less polluted), main basin (moderately polluted) and SW basin (highly polluted), in an attempt to develop sensitive biomarker to evaluate pollution effects from multiple sources. ...
Uchida Yoshikazu - - 2007
Ceramides in mammalian stratum corneum comprise a heterogeneous mixture of molecular species that subserve the epidermal permeability barrier, an essential function for survival in a terrestrial environment. In addition to a variation of sphingol species, hydroxylation of the amide-linked fatty acids contributes to the diversity of epidermal ceramides. Fatty acid ...
Dimmock David - - 2007
The proband was born at 36 weeks, appropriate for gestational age, to nonconsanguineous white parents. There was no evidence of hyperbilirubinemia or intrahepatic cholestasis in the neonatal period, and she had normal newborn screen results. She presented with 3 episodes of life-threatening bleeding and anemia. The diagnostic evaluation for her ...
Saether Thomas - - 2007
On the molecular level, essential fatty acid deficiency (EFAD) has been associated with induced fatty acid (FA) desaturase expression and activity in several tissues. However, there seem to be exceptions. In the present study, we examine the effects of EFAD in the male rat genital tract, combining FA analysis, gene ...
Leonard J V - - 2007
This paper focuses on the three areas in this field in which there have been advances in amino acid and organic acid metabolism. These are the description of glutamine synthetase deficiency, the elucidation of the mechanism of pyridoxine-dependent convulsions, and a hypothesis to explain the neurological complications of some organic ...
Tanito Masaki - - 2007
PURPOSE: To assess the role of vitamin E (VE) in age-related changes in the retinal tissues by using a mouse model of severe VE deficiency. METHODS: Pups of alpha-tocopherol transfer protein null (a-TTP(-)(/)(-)) mice were fed a VE-deficient diet for 4 or 18 months (VE (-) group). Wild-type C57BL/6 mice ...
Chérasse Yoan - - 2007
When an essential amino acid is limited, a signaling cascade is triggered that leads to increased translation of the 'master regulator', activating transcription factor 4 (ATF4), and resulting in the induction of specific target genes. Binding of ATF4 to the amino acid response element (AARE) is an essential step in ...
Atamna Hani - - 2007
Four of the 5 biotin-dependent carboxylases (BDC) are in the mitochondria. BDC replace intermediates in the Krebs [tricarboxylic acid (TCA)] cycle that are regularly removed for the synthesis of key metabolites such as heme or amino acids. Heme, unlike amino acids, is not recycled to regenerate these intermediates, is not ...
Feldhaar Heike - - 2007
Carpenter ants (genus Camponotus) are considered to be omnivores. Nonetheless, the genome sequence of Blochmannia floridanus, the obligate intracellular endosymbiont of Camponotus floridanus, suggests a function in nutritional upgrading of host resources by the bacterium. Thus, the strongly reduced genome of the endosymbiont retains genes for all subunits of a ...
McCue Marshall D - - 2007
Because of the uncertainty in food resources in nature, all animals face the possibility of imposed periods of fasting (i.e., starvation) at some point in their lives. I investigated physiological and biochemical responses to starvation that occur in a species of rattlesnake known to tolerate successfully prolonged periods of starvation ...
Das Undurti N - - 2006
Essential fatty acids (EFAs): cis-linoleic acid (LA) and alpha-linolenic acid (ALA) are essential for humans and their deficiency is rare in humans due to their easy availability in diet. EFAs are metabolized to their respective long-chain metabolites: dihomo-gamma-linolenic acid (DGLA), and arachidonic acid (AA) from LA; and eicosapentaenoic acid (EPA) ...
Mayell S J - - 2007
Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is the commonest disorder of fatty acid metabolism, with a high incidence of morbidity and mortality at presentation. We report a 16 year old girl with first presentation of MCAD deficiency following an alcoholic binge and subsequent period of starvation. Presentation was as acute encephalopathy ...
Bjugstad K B - - 2006
Glutaric acidaemia type I (GA I) is an inborn error of metabolism caused by a deficiency of glutaryl-CoA dehydrogenase (GCDH) and is characterized clinically by striatal degeneration that almost always occurs in early childhood. A murine knockout model of GA I has the organic aciduria seen in the human disorder, ...
Anselm Irina A - - 2006
This report presents the case of an adult male with aromatic L-amino acid decarboxylase deficiency who developed serious cardiac rhythm disturbances during treatment with intravenous dopamine and norepinephrine for severe hypotension. Three weeks later, he spontaneously developed atrial fibrillation while not receiving exogenous catecholamines. He died suddenly after several months. ...
Nakashima Hiroko - - 2006
Necrolytic migratory erythema (NME) is an uncommon inflammatory dermatosis with a distinctive clinical and histological appearance. It shows irregular erythema, bullae, erosion, crusts and pigmentation. While it is typically associated with glucagonoma, some cases of NME without glucagonoma have been reported. Herein, we report a case of necrolytic migratory erythema ...
Raz-Prag Dorit - - 2006
Autosomal dominant Stargardt-like (STGD3) disease results from mutations in the ELOVL4 gene (elongation of very-long-chain fatty acids). This study was undertaken to characterize a mouse model with a targeted deletion of Elovl4 and to explore the role of this gene in retinal/macular degeneration. A construct targeted to exon 2 of ...
van Meeteren Laurens A - - 2006
Autotaxin (ATX), or nucleotide pyrophosphatase-phosphodiesterase 2, is a secreted lysophospholipase D that promotes cell migration, metastasis, and angiogenesis. ATX generates lysophosphatidic acid (LPA), a lipid mitogen and motility factor that acts on several G protein-coupled receptors. Here we report that ATX-deficient mice die at embryonic day 9.5 (E9.5) with profound ...
Uramatsu Mutsumi - - 2007
Prolidase and prolinase activity is known to be enhanced significantly in some diseases. Recently, the effect of amino acids on prolidase and prolinase activity in normal and prolidase-deficient human erythrocytes was investigated. It was reported that both enzymes were enhanced by glycine and alanine in the presence of MnCl(2). Erythrocytes ...
Gronowitz Eva - - 2006
Essential fatty acids (EFA) have proved to be important for normal bone mineral density (BMD) and bone growth in animal studies. Patients with cystic fibrosis often have low serum EFA levels, and low BMD has also been reported in patients with normal anthropometry. The aim of the present study was ...
Lee Ni-Chung - - 2006
Citrin is a mitochondrial membrane aspartate-glutamate carrier, and citrin deficiency causes both hyperammonaemia in adults (adult-onset type II citrullinaemia, CTLN2) and neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD), with metabolic derangements in gluconeogenesis, aerobic glycolysis, urea synthesis, UDP-galactose epimerase activity, and possibly fatty acid synthesis and utilization. Through neonatal ...
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