Gene/Protein Disease Symptom Drug Enzyme Compound
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 58,342 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

After treatment with various chemical and physical agents, flattened or ring-like saccules may occur in the cytoplasm of parietal cells of the gastric glands of several species of mammals. In the current investigation, similar structures appeared after treatment with high dosages of diazo-oxo-norleucine (DON), a glutamine antagonist. A tentative sequence for their formation is suggested. Saccules formed of unit membrane became abundant in some parietal cells of the treated mice. Single saccules often had narrow lumens and peripheral distensions. The saccules, either singular or several stacked together, became progressively more curved, enclosing a region of cytoplasm that often contained glycogen-like particles and occasionally vesicles or other organelles. Many of the concentric saccules were close to an intracellular canaliculus. Membrane bound cytoplasm containing glycogen-like particles occasionally occurred in the canaliculi, suggesting that exocytosis had occurred. Cytochemistry revealed that glycoproteins were associated with the concentric saccules, probably located on the luminal surface. The glycogen-like particles in all locations stained in a manner characteristic of glycogen. It is suggested that the concentric saccules may form from vesicles of the tubulovesicular system.
Anat Rec 1979 Apr
PMID:Formation of concentric saccules in murine parietal cells after injection of diazo-oxo-norleucine. 21 77

Tubular structures were observed in surface epithelial cells of mice that had been injected with high dosages of diazo-oxo-norleucine (DON), a glutamine antagonist. The tubules often occurred in bundles which contained a variable number of tubules, often as many as one hundred being present. Within the bundles, the tubules were oriented either randomly or parallel to one another. They measured 25 to 35 nm in diameter with angular or circular profiles and were as long as 1 to 2 micron. In the center of each tubule, a small tubule-like component was evident that measured 5 to 7 nm in diameter. With the exception of endoplasmic reticulum, often with attached ribosomes, organelles were excluded from the bundles. Since the tubules and the endoplasmic reticulum occasionally were observed to be continuous, it is suggested that the tubules may originate from this organelle.
Anat Rec 1980 Aug
PMID:The occurrence of an unusual tubular organelle in surface epithelial cells of the mouse ascending colon after injection of diazo-oxo-norleucine. 721 94

Tubular structures were observed in surface epithelial cells of mice that had been injected with high dosages of diazo-oxo-norleucine (DON), a glutamine antagonist. The tubules often occurred in bundles which contained a variable number of tubules, often as many as one hundren being present. Within the bundles, the tubules were oriented either randomly or parallel to one another. They measured 25 to 35 nm in diameter with angular or circular profiles and were as long as 1 to 2 micron. In the center of each tubule, a smaller tubule-like component was evident that measured 5 to 7 nm in diameter. With the exception of endoplasmic reticulum, often with attached ribosomes, organelles were excluded from the bundles. Since the tubules and the endoplasmic reticulum occasionally were observed to be continuous, it is suggested that the tubules may originate from this organelle.
Anat Rec 1980
PMID:The occurrence of an unusual tubular organelle in surface epithelial cells of the mouse ascending colon after injection of diazo-oxo-norleucine. 739 31

Human and ungulate embryos can catabolize amino acids for energy production, whereas rodent embryos cannot, raising the question whether studies of rodent model systems are suitable for extrapolation to the human situation. Therefore, we investigated the expression of the amino acid- and ammonia-metabolizing enzymes glutaminase, glutamate dehydrogenase, glutamine synthase, carbamoylphosphate synthase, and arginase immunohistochemically in a graded series of human embryos and fetuses. During human development the expression of these enzymes is first seen in the liver, then in the mesonephric kidney, and finally in the small intestine. Such a simultaneous expression of nitrogen-metabolizing enzymes was not seen in any other organ. The early appearance of the enzymes involved in amino acid and ammonia metabolism in the human liver, compared to, for example, the rat liver, suggests that catabolism of amino acids may provide an important supply of metabolic energy for the human embryo. The coexpression of glutaminase, glutamate dehydrogenase, and carbamoylphosphate synthase, but not of arginase, in the mesonephros and the small intestine suggests that these organs are involved in the biosynthesis of intermediates of the ornithine cycle, e.g., arginine or citrulline. From a comparison of the developmental appearance of ornithine cycle enzymes in different mammalian species we postulate that an early appearance of these enzymes is generally associated with a relatively slow prenatal growth rate and the use of amino acids as metabolic fuel.
Anat Rec 1994 Apr
PMID:Expression patterns of ammonia-metabolizing enzymes in the liver, mesonephros, and gut of human embryos and their possible implications. 819 45

The incidence of natural scrapie in sheep is associated with polymorphisms of the PrP gene, particularly those at codons 136, 154 and 171. In many breeds, the PrP allele encoding valine at codon 136 confers an extremely high risk of scrapie, but in Suffolk sheep this allele is vanishingly rare. In this study of a single closed flock of Suffolk sheep in Scotland, scrapie occurred primarily in animals which were homozygous for glutamine at codon 171, a genotype which was significantly less frequent in healthy flockmates. However, the apparent linkage between glutamine at codon 171 and scrapie was not completely recessive because two of 64 scrapie cases were heterozygous glutamine/arginine. These results suggest that breeding for increased resistance to scrapie in Suffolks by the selection of animals according to their PrP genotype is a feasible option.
Vet Rec 1997 Jan 18
PMID:Association between natural scrapie and PrP genotype in a flock of Suffolk sheep in Scotland. 902 5

To compare steady-state glutamine turnover using nitrogen, carbon, and hydrogen tracers and to test the validity of monocompartmental equations to determine plasma glutamine turnover under non-steady-state conditions, we infused 10 normal postabsorptive volunteers simultaneously with [3,4-3H]glutamine, [2-15N]glutamine, and [U-14C]glutamine for 4 h to isotopic steady state. Eight of the ten subjects were subsequently infused in a stepwise fashion with exogenous glutamine. Plasma glutamine enrichment and specific activities fit a monoexponential model well (r = 0.89, 0.92, and 0.92 for [2-15N]-, [U-14C]-, and [3,4-3H]glutamine, respectively). Volumes of distribution for each tracer (362 +/- 58, 433 +/- 51, and 446 +/- 63 ml/kg) and the transfer rate constants (0.0224 +/- 0.0020, 0.0222 +/- 0.0020, and 0.0240 +/- 0.0023 min(-1)) for [2-15N]-, [U-14C]-, and [3,4-3H]glutamine, respectively, were not significantly different from one another. However, turnover of glutamine determined with [3,4-3H]glutamine (6.14 +/- 0.54 micromol x kg(-1) x min(-1)) exceeded that determined with [U-14C]glutamine (5.72 +/- 0.541 micromol x kg(-1) x min(-1); P < 0.03), which in turn exceeded that determined with [2-15N]glutamine (4.67 +/- 0.39 micromol x kg(-1) x min(-1), P < 0.01). The monocompartmental non-steady-state equations of both DeBodo et al. (DeBodo, R., R. Steele, A. Dunn, and J. Bishop. Rec. Prog. Horm. Res. 19: 445-448, 1963) and Finegood et al. (Finegood, D., R. Bergman, and M. Vranic. Diabetes 36: 914-924, 1987) yielded acceptable approximations of predicted rates of glutamine plasma appearance with deviations from predicted rates from 0.2 to 1.6% (Finegood et al.) and from 0.1 to 8.2% (DeBodo et al.). Use of a 0.75 pool fraction most closely approximated predicted rates.
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PMID:Steady-state and non-steady-state measurements of plasma glutamine turnover in humans. 914 83

Natural scrapie in sheep is associated with polymorphisms of the PrP gene, particularly at amino acid codons 136, 154 and 171. This paper reports the results of nine scrapie case-control studies in Bleu du Maine, Herdwick, Merino x Shetland, Poll Dorset, Scottish Halfbred, Shetland, Soay, Suffolk and Swaledale sheep from British flocks affected by scrapie. In some outbreaks, scrapie was found to occur only in animals with at least one PrP allele encoding valine at codon 136 (V136), usually a relatively rare allele in healthy controls. In other outbreaks, the V136, PrP allele was either not found or was not an absolute prerequisite for scrapie to develop. Although scrapie had a strong tendency to affect sheep with PrP genotypes homozygous for glutamine at codon 171 (QQ171), these genotypes (QQ171 but varying at other codon positions) were relatively common in healthy controls. The reliable prediction of scrapie susceptibility in previously uninvestigated sheep flocks will therefore require information at least about PrP genotypes at codons 136 and 171.
Vet Rec 1997 Aug 09
PMID:Natural scrapie and PrP genotype: case-control studies in British sheep. 928 41

Pyridoxal 5'-phosphate-dependent aminotransferases reversibly catalyzes the transamination reaction in which the alpha-amino group of amino acid 1 is transferred to the 2-oxo acid of amino acid 2 (usually 2-oxoglutarate) to produce the 2-oxo acid of amino acid 1 and amino acid 2 (glutamate). An aminotransferase must thus be able to recognize and bind two kinds of amino acids (amino acids 1 and 2), the side chains of which are different in shape and properties, from among many other small molecules. The dual substrate recognition mechanism has been discovered based on three-dimensional structures of aromatic amino acids, histidinol phosphate, glutamine:phenylpyruvate, acetylornithine, and branched-chain amino acid aminotransferases. There are two representative strategies for dual substrate recognition. An aromatic amino acid aminotransferase prepares charged and neutral pockets for acidic and aromatic side chains, respectively, at the same place by a large-scale rearrangement of the hydrogen-bond network caused by the induced fit. In a branched-chain aminotransferase, the same hydrophobic cavity implanted with hydrophilic sites accommodates both hydrophobic and acidic side chains without side-chain rearrangements of the active-site residues, which is reminiscent of the lock and key mechanism. Dual substrate recognition in other aminotransferases is attained by combining the two representative methods.
Chem Rec 2005
PMID:Dual substrate recognition of aminotransferases. 1588 12

Diagnostic analyses often employ single antibody systems but are potentially limited by epitope sequence variation. United States regulatory testing for scrapie primarily uses antibody F99/97.6.1 for immunohistochemistry (IHC) of the prion protein associated with scrapie (PrP(Sc)). Whereas the epitope bound by F99/97.6.1 is highly conserved in sheep, a polymorphism in caprine PRNP results in a glutamine to lysine change at codon 222 and affects PrP detection. This study evaluated the performance of immunoassays (Western blot and IHC) in the presence of PRNP polymorphisms observed in U.S. goat populations. Effects of naturally occurring caprine prion protein alterations at codons 142, 143, 146, 154, or 222 were first evaluated using bacterially expressed recombinant normal cellular prion protein (rec-PrP(C)) and commercially available antibodies (F99/97.6.1, F89/160.1.5, L42, and SAF84). Detection of rec-PrP(C) using F89/160.1.5 was reduced by alterations at 142 and 143; this was also observed in brain PrP(C) from goats expressing these PRNP variants. Effect of allelic variation at 222 was confirmed by Western blot with F99/97.6.1. No differences were observed with L42 or SAF84. IHC of brain demonstrated reduced signal with F89/160.1.5 in animals heterozygous at 143. Decreasing F89/160.1.5 titers were used to demonstrate the impact of PrP(Sc) immunolabeling in preclinical goats and as a surrogate for F99/97.6.1 detection in 222 variants. In the absence of epitope-relevant knowledge of individual goat PRNP, a multi-antibody approach or an antibody that binds an invariant site may provide a more robust immunoassay of PrP(Sc) in classical scrapie, thus reducing the likelihood of false-negative results due to allelic variation.
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PMID:PRNP variants in goats reduce sensitivity of detection of PrP(Sc) by immunoassay. 2603 81