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  • 1
    ISSN: 1432-1777
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract. Long-chain acyl-CoA dehydrogenase (LCAD) is one of four enzymes involved in the initial step of mitochondrial β-oxidation of straight-chain fatty acids. It is a member of the acyl-CoA dehydrogenase (Acad or ACAD) gene family of enzymes, which also includes very-long-chain (VLCAD), medium-chain (MCAD), and short-chain (SCAD) acyl-CoA dehydrogenases. These enzymes all have similar activity but differ only in the chain length specificity for their substrate. Mitochondrial β-oxidation provides an important source of energy especially during times of fasting. In order to understand the role of LCAD in this pathway, we have cloned and characterized the entire mouse (Mus musculus) gene encoding LCAD (Acadl). Acadl is a single-copy, nuclear encoded gene approximately 35 kb in size. We have sequenced the entire coding region, all intron/exon boundaries, 1.7 kb of its 5′ regulatory region, and mapped the transcription start site. The gene contains 11 coding exons ranging in size from 67 bp to 275 bp, interrupted by 10 introns ranging in size from 1.0 kb to 6.6 kb in size. The Acadl 5′ regulatory region, like other members of the Acad family, lacks a TATA or CAAT box and is GC rich. This region does contain multiple, putative cis-acting DNA elements recognized by either SP1 or members of the steroid-thyroid family of nuclear receptors, which has been shown with other members of the ACAD gene family to be important in regulated expression. The characterization of the mouse Acadl gene will allow further study of LCAD in an in vivo model, and how its expression may be coordinated with other members of the Acad gene family.
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  • 2
    Electronic Resource
    Electronic Resource
    [s.l.] : Nature Publishing Group
    Nature 348 (1990), S. 280-280 
    ISSN: 1476-4687
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
    Notes: [Auszug] SIR-P. R. Sheldon (Nature 347, 704; 25 October 1990) asks why simple organisms such as bacteria do not develop the same complexity as other less numerous lineages, even though their prolific numbers should allow more variations to develop. The simple answer is that eukaryotic organisms have more ...
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  • 3
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] In rodents, the electroencephalogram (EEG) during paradoxical sleep and exploratory behavior is characterized by theta oscillations. Here we show that a deficiency in short-chain acyl-coenzyme A dehydrogenase (encoded by Acads) in mice causes a marked slowing in theta frequency during paradoxical ...
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  • 4
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract: The biochemical basis of a case of GM2 gangliosidosis in a Japanese Spaniel was studied. This dog had a massive accumulation of GM2 ganglioside in the brain. The β-hexosaminidase activity in this affected dog brain was ∼ 12 times higher than that of normal brain. However, the activity toward p-nitrophenyl-6-sulfo-2-acetamido-2-de-oxyglucopyranoside was only four times higher in the affected brain than in normal brain. The GM2 activator preparation obtained from the normal dog brain could stimulate the hydrolysis of GM2 ganglioside by β-hexosaminidase isolated from the affected dog. However, the corresponding activator fraction from the affected dog could not stimulate such a reaction. It was concluded that the biochemical basis of the Gm2 gangliosidosis in this Japanese Spaniel was due to the attenuation in the stimulatory activity of GM2 activator. This case represents the first animal form similar to the activator deficiency (or defect) of Type AB GM2 gangliosidosis in humans.
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  • 5
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract: Neurochemical studies were performed on synaptosomal membranes from cats with GM1 or GM2 gangliosidosis to examine possible mechanisms of neuronal dysfunction in these disorders. The basic hypothesis tested was that deficient ganglioside catabolism causes increased ganglioside content of synaptosomal plasma membrane which in turn disrupts normal function. Fluidity characteristics of synaptosomal membranes were examined using fluorescence polarization. Results showed markedly reduced membrane fluidity in both GM1 and GM2 gangliosidosis. These results were supported by a second study which revealed that isolated synaptosomal membranes of GM1 gangliosidosis cats had a 24-fold increase in total ganglioside content caused predominantly by excess GM1, a 2.3-fold increased cholesterol content, and a 1.4-fold increased phospholipid content. Finally, kinetic analysis of synaptosomal plasma membrane Na+, K+-ATPase from cats with GM1 gangliosidosis showed negligible differences in kinetic parameters compared with controls. Thus, the enzyme appeared protected from the global membrane changes in fluidity and composition. These observations provide evidence for a pathogenetic mechanism of neuronal dysfunction in the gangliosidoses while demonstrating protection of certain vital functional components, such as Na+, K+-ATPase.
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  • 6
    ISSN: 1432-1777
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract. Mitochondrial β-oxidation of long-chain fatty acids (LCFA) is essential for mammalian life. Because portions of this metabolic pathway are composed of enzymes that are coordinately regulated and share structural and functional similarities, we evaluated five of these enzyme genes for possible chromosomal linkages. Regulation of LCFA catabolism influences cell signal pathways and apoptosis, as well as energy production from LCFA. Partial cDNA fragments of the mouse mitochondrial proteins carnitine acetyltransferase (Crat), very-long-chain acyl coenzyme A dehydrogenase (Acadvl), the liver and muscle isoforms of carnitine acyltransferase I (Cpt1a and Cpt1b respectively), and a genomic PCR product of mitochondrial protein carnitine acyltransferase II (Cpt2) were used in a previously established mapping panel to determine their chromosomal locations. No pseudogenes were detected for any of the genes in Mus musculus, and all of the genes mapped to different chromosome locations, including the tissue-specific isoforms of carnitine palmitoyltransferase. Crat mapped to Chromosome (Chr) 2, at a position approximately 18 cM from the centromere and 2 cM proximal to the gene Ass1. Acadvl mapped to the middle of Chr 11, 8.3 cM distal to Il4 and 2.8 cM proximal to Mpmv2. Cpt1a mapped to the centromeric region of Chr 19, 8.7 cM proximal to Pomc-ps1. Cpt1b mapped to Chr 15, 4.9 distal to Gpt1 and 3.5 cM proximal to Wnt1. Cpt2 mapped to Chr 4 near the locus Pmv19.
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  • 7
    Electronic Resource
    Electronic Resource
    Hoboken, NJ : Wiley-Blackwell
    AIChE Journal 37 (1991), S. 448-460 
    ISSN: 0001-1541
    Keywords: Chemistry ; Chemical Engineering
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology , Process Engineering, Biotechnology, Nutrition Technology
    Notes: Efforts to model the turbulent flow in stirred tanks require accurate boundary conditions at the tip of the impeller, not just of velocities, but of the turbulence quantities k and ε. Kolar's (1982) phenomenological, swirling radial jet model of the impeller region is extended by using a two-equation k - ε turbulence model to obtain direct estimates of k and ε on the impeller periphery. The model is extended and clarified, so that the number of parameters required for its application is reduced to two: the rotational speed and the diameter of the impeller. Three-dimensional simulations allow a realistic treatment of the baffles. Agreement of the modeling results with recently published experimental data is excellent. This is particularly true in the important impeller discharge zone, where details of the predicted behavior of the turbulence kinetic energy and dissipation rate are in quantitative agreement with the available data. Based on these results, average values of ε are calculated, along with the zones over which the apply. For the impeller discharge zone, the dimensionless, volume-averaged ε is 0.19.
    Additional Material: 13 Ill.
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  • 8
    ISSN: 0021-8995
    Keywords: Chemistry ; Polymer and Materials Science
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology , Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics , Physics
    Notes: The plasticizing effect of n-pentane on the rate of bulk free radical polymerization of styrene and molecular weight distribution development has been modeled on the basis of the free volume theory for both monofunctional and bifunctional initiation. A strong decrease in the reaction rate in the late stages of the polymerization, due to the displacement of the onset of the gel effect, has been observed for both types of initiation. This decrease in the polymerization rate limited the terminal conversion to values well below 100% for mono-functional initiation. However, in bifunctionally initiated polymerization, terminal conversions close to 100% were obtained in spite of the decrease in reaction rate. Contrary to what was expected, the molecular weight distribution obtained at terminal conversion was almost completely insensitive to these changes in polymerization rate. This phenomenon is explained in terms of limited transfer to monomer reactions when n-pentane is present in the system. In suspension polymerization, the limiting conversion and plasticizing effects of n-pentane in monofunctionally initiated systems, caused enhanced coalescence leading to suspension set-up. In bifunctionally initiated systems this enhanced coalescence was completely overcome by the short duration of the particle growth stage, owing to high polymerization rates, and stable suspensions were achieved. For these systems the particle size distributions obtained were similar to that of suspension polystyrene without n-pentane. © 1993 John Wiley & Sons, Inc.
    Additional Material: 11 Ill.
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  • 9
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] The molecular basis of X-linked recessive anhidrotic ectodermal dysplasia with immunodeficiency (EDA-ID) has remained elusive. Here we report hypomorphic mutations in the gene IKBKG in 12 males with EDA-ID from 8 kindreds, and 2 patients with a related and hitherto unrecognized syndrome of EDA-ID ...
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  • 10
    ISSN: 1572-9931
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract As part of an effort to develop a model system for somatic gene therapy, a human argininosuccinate synthetase (AS) cDNA has been introduced into two gag+ retroviral vectors, and high titer clones were obtained for both constructs. The presence of proviral DNA sequences was detected in individual spleen colonies after infection of primary murine marrow cells with each virus. Mice were reconstituted for long-term survival with marrow infected with one virus, and they demonstrated elevated levels of AS expression in peripheral blood for up to eight weeks posttransplantation.
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