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Ajit S Divakaruni et al.
Analytical biochemistry, 552, 60-65 (2017-10-11)
Activities of enzymes localized to the mitochondrial matrix of mammalian cells are often critical regulatory steps in cellular metabolism. As such, measurement of matrix enzyme activities in response to genetic modifications or drug interventions is often desired. However, measurements in
Nayla Munawar et al.
Extremophiles : life under extreme conditions, 16(3), 463-476 (2012-04-25)
Enzymes produced by halophilic archaea are generally heat resistant and organic solvent tolerant, and accordingly important for biocatalytic applications in 'green chemistry', frequently requiring a low-water environment. NAD(+)-dependent glutamate dehydrogenase from an extremely halophilic archaeon Halobacterium salinarum strain NRC-36014 was
Andreas Plaitakis et al.
Neurochemistry international, 59(4), 495-509 (2011-03-23)
Whereas glutamate dehydrogenase in most mammals (hGDH1 in the human) is encoded by a single functional GLUD1 gene expressed widely, humans and other primates have acquired through retroposition an X-linked GLUD2 gene that encodes a highly homologous isoenzyme (hGDH2) expressed
The crystallization and characterization of L-glutamic acid dehydrogenase.
J A OLSON et al.
The Journal of biological chemistry, 197(1), 67-79 (1952-05-01)
Flavio Faletra et al.
Gene, 521(1), 160-165 (2013-03-20)
Congenital hyperinsulinism (CHI) is a genetic disorder characterized by profound hypoglycemia related to an inappropriate insulin secretion. It is a heterogeneous disease classified into two major subgroups: "channelopathies" due to defects in ATP-sensitive potassium channel, encoded by ABCC8 and KCNJ11
Virginia Kroef et al.
eLife, 11 (2022-03-02)
The hexosamine biosynthetic pathway (HBP) produces the essential metabolite UDP-GlcNAc and plays a key role in metabolism, health, and aging. The HBP is controlled by its rate-limiting enzyme glutamine fructose-6-phosphate amidotransferase (GFPT/GFAT) that is directly inhibited by UDP-GlcNAc in a
Glutamic dehydrogenase.
H J STRECKER
Archives of biochemistry and biophysics, 46(1), 128-140 (1953-09-01)
Generalized dystonia in a patient with a novel mutation in the GLUD1 gene.
Ryosuke Miyamoto et al.
Movement disorders : official journal of the Movement Disorder Society, 27(9), 1198-1199 (2012-06-26)
Ludmila A Kasatkina et al.
The international journal of biochemistry & cell biology, 119, 105665-105665 (2019-12-11)
Vitamin D3 is among the major neurosteroids whose role in developing and adult brain is intensively studied now. Its active form 1,25(OH)2D3 regulates the expression and functioning of a range of brain-specific proteins, which orchestrate the neurotransmitter turnover, neurogenesis and
S Vesce et al.
FEBS letters, 411(1), 107-109 (1997-07-07)
The mechanisms of HIV-1 neurotoxicity remain still undefined although the induction of signalling events and a modest inhibition of glutamate uptake induced by the envelope glycoprotein, gp120, have called attention to astrocytes. Here we demonstrate that the levels at which
Louise Fets et al.
Nature chemical biology, 14(11), 1032-1042 (2018-10-10)
α-Ketoglutarate (αKG) is a key node in many important metabolic pathways. The αKG analog N-oxalylglycine (NOG) and its cell-permeable prodrug dimethyloxalylglycine (DMOG) are extensively used to inhibit αKG-dependent dioxygenases. However, whether NOG interference with other αKG-dependent processes contributes to its
Feng Cheng et al.
Biotechnology and bioengineering, 116(9), 2156-2166 (2019-05-08)
Arginine deiminase (ADI) is a therapeutic protein for cancer therapy of arginine-auxotrophic tumors. However, its application as anticancer drug is hampered by its poor stability under physiological conditions in the bloodstream. Commonly, random PEGylation is being used for increasing the
Mehran Karimi et al.
Seminars in thrombosis and hemostasis, 35(4), 426-438 (2009-07-15)
Factor XIII (FXIII) is a tetrameric zymogen (FXIII-A (2)B (2)) that is converted into an active transglutaminase (FXIIIa) by thrombin and Ca (2+) in the terminal phase of the clotting cascade. By cross-linking fibrin chains and alpha (2) plasmin inhibitor
Manish Verma et al.
Biochimica et biophysica acta, 1827(1), 38-49 (2012-10-10)
The sustained opening of the mitochondrial permeability transition pore (PTP) is a decisive event in the onset of irreversible cell injury. The PTP is modulated by numerous exogenous and endogenous effectors, including mitochondrial membrane potential, ions and metabolites. Mitochondrial sirtuins
Hyperinsulinism and hyperammonaemia syndrome and severe myoclonic epilepsy of infancy.
F Pérez Errazquin et al.
Neurologia (Barcelona, Spain), 26(4), 248-252 (2010-12-18)
Ludmila A Kasatkina et al.
Biochemical pharmacology, 174, 113783-113783 (2019-12-28)
Neuroinflammation plays a prominent role in the onset of demyelinating diseases, major depressive disorder and delayed neurodegeneration. An open question remains whether pharmacological suppression of inflammation can effectively reduce the progression of these states. Bioactive lipid mediators such as N-acylethanolamines
Xiaoguang Wang et al.
Archives of microbiology, 201(4), 519-530 (2018-11-09)
Nitrate and nitrite reduction are of paramount importance for nitrogen assimilation and anaerobic metabolism, and understanding the specific roles of each participating reductase is necessary to describe the biochemical balance that dictates cellular responses to their environments. The soluble, cytoplasmic
Konstantinos Kanavouras et al.
Journal of neurochemistry, 109 Suppl 1, 167-173 (2009-05-07)
Glutamate dehydrogenase (GDH) in human exists in GLUD1 and GLUD2 gene-encoded isoforms (hGDH1 and hGDH2, respectively), differing in their regulation and tissue expression pattern. Whereas hGDH1 is subject to GTP control, hGDH2 uses for its regulation, a novel molecular mechanism
Mariagrazia Grimaldi et al.
Human molecular genetics, 26(18), 3453-3465 (2017-09-16)
Congenital hyperinsulinism/hyperammonemia (HI/HA) syndrome gives rise to unregulated protein-induced insulin secretion from pancreatic beta-cells, fasting hypoglycemia and elevated plasma ammonia levels. Mutations associated with HI/HA were identified in the Glud1 gene, encoding for glutamate dehydrogenase (GDH). We aimed at identifying
Ioannis Zaganas et al.
Neurochemistry international, 61(4), 455-462 (2012-06-20)
Glutamate dehydrogenase (GDH), a mitochondrial enzyme with a key metabolic role, exists in the human in hGDH1 and hGDH2 isoforms encoded by the GLUD1 and GLUD2 genes, respectively. It seems that GLUD1 was retroposed to the X chromosome where it
Ioannis Zaganas et al.
Neurochemistry international, 55(1-3), 52-63 (2009-05-12)
In all mammals, glutamate dehydrogenase (GDH), an enzyme central to the metabolism of glutamate, is encoded by a single gene (GLUD1 in humans) which is expressed widely (housekeeping). Humans and other primates also possess a second gene, GLUD2, which encodes
Joaquín V Rodriguez et al.
Artificial organs, 32(4), 323-328 (2008-03-29)
This work deals with the construction and performance of a hollow fiber-based minibioreactor (MBR). Due to its simple design and the utilization of standard materials, it could serve as a suitable tool to evaluate the behavior and performance of cold
Charles A Stanley
Neurochemistry international, 59(4), 465-472 (2010-12-07)
Glutamate dehydrogenase (GDH) has recently been shown to be involved in two genetic disorders of hyperinsulinemic hypoglycemia in children. These include the hyperinsulinism/hyperammonemia syndrome caused by dominant activating mutations of GLUD1 which interfere with inhibitory regulation by GTP and hyperinsulinism
Dimitra Kotzamani et al.
Neurochemistry international, 61(4), 463-469 (2012-06-20)
Human glutamate dehydrogenase (hGDH) exists in two highly homologous isoforms with a distinct regulatory and tissue expression profile: a housekeeping hGDH1 isoprotein encoded by the GLUD1 gene and an hGDH2 isoenzyme encoded by the GLUD2 gene. There is evidence that
Anna K Junk et al.
Molecular vision, 16, 1286-1291 (2010-07-29)
To determine whether activity of carbohydrate metabolism enzymes (aldolase, pyruvate kinase, isocitrate dehydrogenase, and malate dehydrogenase) are altered in the glaucomatous trabecular meshwork (TM) compared to controls. Tissue specimens were obtained from trabeculectomy (n=45 open angle glaucoma; Caucasian, average age
Cleanthe Spanaki et al.
Neurochemistry international, 61(4), 470-481 (2012-06-05)
Mammalian glutamate dehydrogenase (GDH) is a housekeeping mitochondrial enzyme (hGDH1 in the human) that catalyses the reversible inter-conversion of glutamate to α-ketoglutarate and ammonia, thus interconnecting amino acid and carbohydrate metabolism. It displays an energy sensing mechanism, which permits enzyme
Cleanthe Spanaki et al.
Neurotoxicity research, 21(1), 117-127 (2011-11-01)
Glutamate dehydrogenase (GDH) catalyzes the reversible inter-conversion of glutamate to α-ketoglutarate and ammonia. High levels of GDH activity is found in mammalian liver, kidney, brain, and pancreas. In the liver, GDH reaction appears to be close-to-equilibrium, providing the appropriate ratio
Jaekyoung Son et al.
Nature, 496(7443), 101-105 (2013-03-29)
Cancer cells have metabolic dependencies that distinguish them from their normal counterparts. Among these dependencies is an increased use of the amino acid glutamine to fuel anabolic processes. Indeed, the spectrum of glutamine-dependent tumours and the mechanisms whereby glutamine supports
Lia Rosso et al.
PLoS genetics, 4(8), e1000150-e1000150 (2008-08-09)
Many new gene copies emerged by gene duplication in hominoids, but little is known with respect to their functional evolution. Glutamate dehydrogenase (GLUD) is an enzyme central to the glutamate and energy metabolism of the cell. In addition to the
Mohammad Mehdi Maneshi et al.
Scientific reports, 7, 39610-39610 (2017-01-04)
While studying the physiological response of primary rat astrocytes to fluid shear stress in a model of traumatic brain injury (TBI), we found that shear stress induced Ca2+ entry. The influx was inhibited by MK-801, a specific pore blocker of
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