909 research outputs found

    Aquaculture Industry of Arkansas in 1979-1980

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    A survey of previous fish farmer certificate holders in Arkansas was conducted during 1979-1980 using renewal questionnaires, telephone conversations, and personal contacts. This survey was compared with similar surveys from preceding years. Approximately 51.0% of 12,372 intensively farmed hectares in 1979-80 were devoted to bait fish production, while 22.9% were utilized in food fish production. Acreage in bait fish, food fish, and fingerling production decreased from 1979 to 1980; however, price increases during this time resulted in a higher total value of the industr

    Sweet solution: sugars to the rescue

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    Sugar pills are usually placebos, but Smith et al. (2002, this issue) use one to rescue designer mice unable to make GDP-Fucose. Dietary fucose enters a salvage pathway and spares the mice. Sound simple? Not so. Unknown genetic factors determine life or death

    Molecular basis of carbohydrate-deficient glycoprotein syndromes type I with normal phosphomannomutase activity

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    AbstractCarbohydrate deficient glycoprotein syndromes (CDGS) are inherited disorders in glycosylation. Isoelectric focusing of serum transferrin is used as a biochemical indicator of CDGS; however, this technique cannot diagnose the molecular defect. Even though phosphomannomutase (PMM) deficiency accounts for the great majority of known CDGS cases (CDGS type Ia), newly discovered cases have significantly different clinical presentations than the PMM-deficient patients. These differences arise from other defects affecting the biosynthesis of N-linked oligosaccharides in the endoplasmic reticulum and in the Golgi compartment. The most notable is the loss of phosphomannose isomerase (PMI) (CDGS type Ib). It causes severe hypoglycemia, protein-losing enteropathy, vomiting, diarrhea, and congenital hepatic fibrosis. In contrast to PMM-deficiency, there is no developmental delay nor neuropathy. Most symptoms in the PMI-deficient patients can be successfully treated with dietary mannose supplements. Another defect is the lack of glucosylation of the lipid-linked oligosaccharide precursor. The clinical features of this form of CDGS are milder, but similar to, PMM-deficient patients. Yeast genetic and biochemical techniques were critical in unraveling these disorders since many of the defective genes were known in yeast and corresponding mutants were available for complementation. Yeast strains carrying mutations in the homologous genes are likely to provide conclusive identification of the primary defects in novel CDGS types that affect the synthesis and transfer of precursor oligosaccharides

    The congenital disorders of glycosylation: A multifaceted group of syndromes

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    Application of Random Walk Model for Timing Recovery in Modern Mobile SATCOM Systems

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    In a modern mobile satellite communication (SATCOM) system, a ground terminal receiver receives a radio frequency signal that is demodulated to generate a baseband digital signal waveform containing a self-clocking bit stream of digital data. The received baseband digital signal waveform is recovered and tracked using a timing recovery loop (TRL). The traditional TRLs use early-and-late gates, digital transition tracking, filter-and-square, and delay-and-multiply functions. In bit timing detection, the bit stream is self-clocking and the timing differential dithers about correct bit timing in the TRLs. For mobile satellite communication environments, the traditional TRLs drop lock when the loop signal-to-noise ratio (SNR) is smaller than a threshold value or the residual Doppler frequency is larger than the operating loop bandwidth. After dropping lock, the traditional TRLs experience long hang up time due to the need to reacquire the timing pulses. Recently, random walk filters (RWF) have been adapted to improve the bit clock locking stability and are applied to recover bit timing information of a digital data stream. This chapter describes random walk model for timing jitter and discusses how RWF solution can address the timing recovery challenges in mobile satellite communication environments

    Pathogenic Variants in Fucokinase Cause a Congenital Disorder of Glycosylation

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    FUK encodes fucokinase, the only enzyme capable of converting L-fucose to fucose-1-phosphate, which will ultimately be used for synthesizing GDP-fucose, the donor substrate for all fucosyltransferases. Although it is essential for fucose salvage, this pathway is thought to make only a minor contribution to the total amount of GDP-fucose. A second pathway, the major de novo pathway, involves conversion of GDP-mannose to GDP-fucose. Here we describe two unrelated individuals who have pathogenic variants in FUK and who presented with severe developmental delays, encephalopathy, intractable seizures, and hypotonia. The first individual was compound heterozygous for c.667T>C (p.Ser223Pro) and c.2047C>T (p.Arg683Cys), and the second individual was homozygous for c.2980A>C (p.Lys994Gln). Skin fibroblasts from the first individual confirmed the variants as loss of function and showed significant decreases in total GDP-[3H] fucose and [3H] fucose-1-phosphate. There was also a decrease in the incorporation of [5,6-3H]-fucose into fucosylated glycoproteins. Lys994 has previously been shown to be an important site for ubiquitin conjugation. Here, we show that loss-of-function variants in FUK cause a congenital glycosylation disorder characterized by a defective fucose-salvage pathway

    IS Success Model in E-Learning Context Based on Students\u27 Perceptions

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    This study utilized the Information Systems Success (ISS) model in examining e-learning systems success. The study was built on the premise that system quality (SQ) and information quality (IQ) influence system use and user satisfaction, which in turn impact system success. A structural equation model (SEM), using LISREL, was used to test the measurement and structural models using a convenience sample of 674 students at a Midwestern university. The results revealed that both system quality and information quality had significant positive impact on user satisfaction and system use. Additionally, the results showed that user satisfaction, compared to system use, had a stronger impact on system success. Implications for educators and researchers are reported

    On the generalized Davenport constant and the Noether number

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    Known results on the generalized Davenport constant related to zero-sum sequences over a finite abelian group are extended to the generalized Noether number related to the rings of polynomial invariants of an arbitrary finite group. An improved general upper bound is given on the degrees of polynomial invariants of a non-cyclic finite group which cut out the zero vector.Comment: 14 page

    Insufficient ER-stress response causes selective mouse cerebellar granule cell degeneration resembling that seen in congenital disorders of glycosylation

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    BACKGROUND: Congenital disorders of glycosylation (CDGs) are inherited diseases caused by glycosylation defects. Incorrectly glycosylated proteins induce protein misfolding and endoplasmic reticulum (ER) stress. The most common form of CDG, PMM2-CDG, is caused by deficiency in the cytosolic enzyme phosphomannomutase 2 (PMM2). Patients with PMM2-CDG exhibit a significantly reduced number of cerebellar Purkinje cells and granule cells. The molecular mechanism underlying the specific cerebellar neurodegeneration in PMM2-CDG, however, remains elusive. RESULTS: Herein, we report that cerebellar granule cells (CGCs) are more sensitive to tunicamycin (TM)-induced inhibition of total N-glycan synthesis than cortical neurons (CNs). When glycan synthesis was inhibited to a comparable degree, CGCs exhibited more cell death than CNs. Furthermore, downregulation of PMM2 caused more CGCs to die than CNs. Importantly, we found that upon PMM2 downregulation or TM treatment, ER-stress response proteins were elevated less significantly in CGCs than in CNs, with the GRP78/BiP level showing the most significant difference. We further demonstrate that overexpression of GRP78/BiP rescues the death of CGCs resulting from either TM-treatment or PMM2 downregulation. CONCLUSIONS: Our results indicate that the selective susceptibility of cerebellar neurons to N-glycosylation defects is due to these neurons’ inefficient response to ER stress, providing important insight into the mechanisms of selective neurodegeneration observed in CDG patients

    System-of-Systems Enterprise CONOPS Assessment Decision Support Tools

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    This chapter discusses the implementation of System-of-Systems Enterprise Architecture (SOSEA) CONOPS assessment framework and models in Matlab, and presents preliminary results concerning SOSEA resiliency in the presence of a notional Radio Frequency Interference (RFI) scenario. The chapter provides an overview of the SOSEA CONOPS Assessment Framework, and discusses related SOS Resiliency Models including Resilient Assessment Index Against RFI (RAI-RFI), Spectrum Resiliency Assessment Index (SRAI), and Resilient Capacity (RC)
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