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Glucose transporter deficiency

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https://www.readbyqxmd.com/read/28688166/triheptanoin-for-the-treatment-of-brain-energy-deficit-a-14-year-experience
#1
REVIEW
Fanny Mochel
Triheptanoin is an odd-chain triglyceride with anaplerotic properties-that is, replenishing the pool of metabolic intermediates in the Krebs cycle. Unlike even-chain fatty acids metabolized to acetyl-CoA only, triheptanoin can indeed provide both acetyl-CoA and propionyl-CoA, two key carbon sources for the Krebs cycle. Triheptanoin was initially used in patients with long-chain fatty acid oxidation disorders. The first demonstration of the possible benefit of triheptanoin for brain energy deficit came from a patient with pyruvate carboxylase deficiency, a severe metabolic disease that affects anaplerosis in the brain...
July 8, 2017: Journal of Neuroscience Research
https://www.readbyqxmd.com/read/28687021/insulin-resistance-in-vitamin-d-deficient-mice-is-alleviated-by-n-acetylcysteine
#2
Zhao-Hui Cui, Qi Yuan, Li Mao, Feng-Li Chen, Feng Ji, Sha Tao
Vitamin D deficiency will lead to insulin resistance. In the current study, vitamin D3 1α-Hydroxylase ["1α(OH)ase"] knockout mice were generated to mimic vitamin D deficiency in vivo. As compared to the wild-type mice, the liver tissues of the knockout mice showed impaired insulin signaling, decreased glucose transporter 4 expression and increased reactive oxygen species production. Meanwhile, p53-p21 activation, apoptosis intensity and pro-inflammatory cytokines (IL-6, IL-1 and MIP-1α) level were significantly increased in the knockout mice livers...
June 28, 2017: Oncotarget
https://www.readbyqxmd.com/read/28687018/vitamin-d-deficiency-causes-insulin-resistance-by-provoking-oxidative-stress-in-hepatocytes
#3
Sha Tao, Qi Yuan, Li Mao, Feng-Li Chen, Feng Ji, Zhao-Hui Cui
Vitamin D deficiency could cause insulin resistance. However, the underlying mechanisms are unclear. The 1α-Hydroxylase ["1α(OH)ase"] is a key enzyme for activate vitamin D3 synthesis. Here, we show that 1α(OH)ase stable knockdown by targeted shRNA led to vitamin D3 depletion in L02 hepatocytes. 1α(OH)ase silence also inhibited insulin-induced downstream signaling (IRS-1, ERK and AKT) transduction and glucose transporter 4 expression. Further, 1α(OH)ase shRNA in L02 hepatocytes led to significant reactive oxygen species production, p53-p21 activation and DNA damages...
June 28, 2017: Oncotarget
https://www.readbyqxmd.com/read/28674555/a-design-build-test-cycle-using-modeling-and-experiments-reveals-interdependencies-between-upper-glycolysis-and-xylose-uptake-in-recombinant-s-cerevisiae-and-improves-predictive-capabilities-of-large-scale-kinetic-models
#4
Ljubisa Miskovic, Susanne Alff-Tuomala, Keng Cher Soh, Dorothee Barth, Laura Salusjärvi, Juha-Pekka Pitkänen, Laura Ruohonen, Merja Penttilä, Vassily Hatzimanikatis
BACKGROUND: Recent advancements in omics measurement technologies have led to an ever-increasing amount of available experimental data that necessitate systems-oriented methodologies for efficient and systematic integration of data into consistent large-scale kinetic models. These models can help us to uncover new insights into cellular physiology and also to assist in the rational design of bioreactor or fermentation processes. Optimization and Risk Analysis of Complex Living Entities (ORACLE) framework for the construction of large-scale kinetic models can be used as guidance for formulating alternative metabolic engineering strategies...
2017: Biotechnology for Biofuels
https://www.readbyqxmd.com/read/28673577/cognitive-impairment-in-diabetes-and-poor-glucose-utilization-in-the-intracellular-neural-milieu
#5
Carlos M Contreras, Ana G Gutiérrez-García
The main characteristic of diabetes is hyperglycemia. Depending on whether diabetes is type-1 or type-2, it is characterized by deficiencies in insulin secretion, insulin receptor sensitivity, hexokinase activity, and glucose transport. Current drug treatments are able to lower circulating glucose but do not address the problem of glucose utilization in the intracellular milieu, the consequence of which is tissue damage. In the long-term, such changes can produce structural damage in many cortical and subcortical brain areas that are related to cognitive function...
July 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/28666985/keratins-regulate-%C3%AE-cell-mitochondrial-morphology-motility-and-homeostasis
#6
Jonas S G Silvander, Sofie M Kvarnström, Angeli Kumari-Ilieva, Anup Shrestha, Catharina M Alam, Diana M Toivola
Loss of the epithelial intermediate filament protein keratin 8 (K8) in murine β-cells leads to irregular insulin vesicles and decreased insulin levels. Because mitochondria are central in glucose-stimulated insulin secretion, the relationship between keratins and β-cell mitochondrial function and morphology was investigated. β-cells in murine K8 knockout (K8(-/-)) islets of Langerhans have increased numbers of mitochondria, which are rounder and have diffuse cristae, as seen by electron microscopy. The mitochondrial network in primary cultured K8(-/-) β-cells is more fragmented compared with K8(+/+) mitochondria, correlating with decreased levels of mitofusin 2 and the mitofusin 2- and keratin-binding protein trichoplein...
June 30, 2017: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/28653367/significance-of-l-carnitine-for-human-health
#7
REVIEW
María M Adeva-Andany, Isabel Calvo-Castro, Carlos Fernández-Fernández, Cristóbal Donapetry-García, Ana María Pedre-Piñeiro
Carnitine acyltransferases catalyze the reversible transfer of acyl groups from acyl-coenzyme A esters to l-carnitine, forming acyl-carnitine esters that may be transported across cell membranes. l-Carnitine is a wáter-soluble compound that humans may obtain both by food ingestion and endogenous synthesis from trimethyl-lysine. Most l-carnitine is intracellular, being present predominantly in liver, skeletal muscle, heart and kidney. The organic cation transporter-2 facilitates l-carnitine uptake inside cells...
June 26, 2017: IUBMB Life
https://www.readbyqxmd.com/read/28639913/novel-roles-of-folic-acid-as-redox-regulator-modulation-of-reactive-oxygen-species-sinker-protein-expression-and-maintenance-of-mitochondrial-redox-homeostasis-on-hepatocellular-carcinoma
#8
Kun-Goung Lai, Chi-Fen Chen, Chun-Te Ho, Jun-Jen Liu, Tsan-Zon Liu, Chi-Liang Chern
We provide herein several lines of evidence to substantiate that folic acid (or folate) is a micronutrient capable of functioning as a novel redox regulator on hepatocellular carcinoma. First, we uncovered that folate deficiency could profoundly downregulate two prominent anti-apoptotic effectors including survivin and glucose-regulated protein-78. Silencing of either survivin or glucose-regulated protein-78 via small interfering RNA interfering technique established that both effectors could serve as reactive oxygen species sinker proteins...
June 2017: Tumour Biology: the Journal of the International Society for Oncodevelopmental Biology and Medicine
https://www.readbyqxmd.com/read/28620410/transcriptome-analysis-of-differentially-expressed-genes-induced-by-low-and-high-potassium-levels-provides-insight-into-fruit-sugar-metabolism-of-pear
#9
Changwei Shen, Jie Wang, Xiaoqian Shi, Yalong Kang, Changyan Xie, Lirun Peng, Caixia Dong, Qirong Shen, Yangchun Xu
Potassium (K) deficiency is a common abiotic stress that can inhibit the growth of fruit and thus reduce crop yields. Little research has been conducted on pear transcriptional changes under low and high K conditions. Here, we performed an experiment with 7-year-old pot-grown "Huangguan" pear trees treated with low, Control or high K levels (0, 0.4, or 0.8 g·K2O/kg soil, respectively) during fruit enlargement and mature stages. We identified 36,444 transcripts from leaves and fruit using transcriptome sequencing technology...
2017: Frontiers in Plant Science
https://www.readbyqxmd.com/read/28620281/inhibition-of-the-mitochondrial-glutamate-carrier-slc25a22-in-astrocytes-leads-to-intracellular-glutamate-accumulation
#10
Emmanuelle Goubert, Yanina Mircheva, Francesco M Lasorsa, Christophe Melon, Emanuela Profilo, Julie Sutera, Hélène Becq, Ferdinando Palmieri, Luigi Palmieri, Laurent Aniksztejn, Florence Molinari
The solute carrier family 25 (SLC25) drives the import of a large diversity of metabolites into mitochondria, a key cellular structure involved in many metabolic functions. Mutations of the mitochondrial glutamate carrier SLC25A22 (also named GC1) have been identified in early epileptic encephalopathy (EEE) and migrating partial seizures in infancy (MPSI) but the pathophysiological mechanism of GC1 deficiency is still unknown, hampered by the absence of an in vivo model. This carrier is mainly expressed in astrocytes and is the principal gate for glutamate entry into mitochondria...
2017: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/28602638/autophagy-dependent-shuttling-of-tbc1d5-controls-plasma-membrane-translocation-of-glut1-and-glucose-uptake
#11
Srirupa Roy, Andrew M Leidal, Jordan Ye, Sabrina M Ronen, Jayanta Debnath
Autophagy traditionally sustains metabolism in stressed cells by promoting intracellular catabolism and nutrient recycling. Here, we demonstrate that in response to stresses requiring increased glycolytic demand, the core autophagy machinery also facilitates glucose uptake and glycolytic flux by promoting cell surface expression of the glucose transporter GLUT1/Slc2a1. During metabolic stress, LC3(+) autophagic compartments bind and sequester the RabGAP protein TBC1D5 away from its inhibitory interactions with the retromer complex, thereby enabling retromer recruitment to endosome membranes and GLUT1 plasma membrane translocation...
July 6, 2017: Molecular Cell
https://www.readbyqxmd.com/read/28591644/co-expression-of-glucose-6-phosphate-dehydrogenase-and-acyl-coa-binding-protein-enhances-lipid-accumulation-in-the-yeast-yarrowia-lipolytica
#12
Evgeniya Y Yuzbasheva, Elizaveta B Mostova, Natalia I Andreeva, Tigran V Yuzbashev, Ivan A Laptev, Tatiana I Sobolevskaya, Sergey P Sineoky
The oleaginous yeast Yarrowia lipolytica is a convenient model for investigating lipid biosynthesis and for engineering high lipid accumulated strains. In this organism, the pentose phosphate pathway is the major source of NADPH for lipid biosynthesis. Thus, we over-expressed gene encoding NADP(+)-dependent glucose-6-phosphate dehydrogenase (ZWF1) in a strain deficient in peroxisome biogenesis. However, this strategy suppressed growth during cultivation under lipogenic conditions and did not significantly increase lipid accumulation...
June 4, 2017: New Biotechnology
https://www.readbyqxmd.com/read/28591637/amino-acid-transporter-slc38a5-controls-glucagon-receptor-inhibition-induced-pancreatic-%C3%AE-cell-hyperplasia-in-mice
#13
Jinrang Kim, Haruka Okamoto, ZhiJiang Huang, Guillermo Anguiano, Shiuhwei Chen, Qing Liu, Katie Cavino, Yurong Xin, Erqian Na, Rachid Hamid, Joseph Lee, Brian Zambrowicz, Roger Unger, Andrew J Murphy, Yan Xu, George D Yancopoulos, Wen-Hong Li, Jesper Gromada
Glucagon supports glucose homeostasis by stimulating hepatic gluconeogenesis, in part by promoting the uptake and conversion of amino acids into gluconeogenic precursors. Genetic disruption or pharmacologic inhibition of glucagon signaling results in elevated plasma amino acids and compensatory glucagon hypersecretion involving expansion of pancreatic α cell mass. Recent findings indicate that hyperaminoacidemia triggers pancreatic α cell proliferation via an mTOR-dependent pathway. We confirm and extend these findings by demonstrating that glucagon pathway blockade selectively increases expression of the sodium-coupled neutral amino acid transporter Slc38a5 in a subset of highly proliferative α cells and that Slc38a5 controls the pancreatic response to glucagon pathway blockade; most notably, mice deficient in Slc38a5 exhibit markedly decreased α cell hyperplasia to glucagon pathway blockade-induced hyperaminoacidemia...
June 6, 2017: Cell Metabolism
https://www.readbyqxmd.com/read/28582795/another-case-of-glucose-transporter-1-deficiency-syndrome-with-periventricular-calcification-cataracts-hemolysis-and-pseudohyperkalemia
#14
Takashi Shibata, Katsuhiro Kobayashi, Harumi Yoshinaga, Hiroaki Ono, Michiko Shinpo, Kuriko Kagitani-Shimono
No abstract text is available yet for this article.
June 5, 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28582793/anemia-in-glucose-transporter-type-1-deficiency-syndrome-often-expected-rarely-encountered-and-with-a-fascinating-explanation
#15
Michèl A Willemsen
No abstract text is available yet for this article.
June 5, 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28572880/effect-of-medicinal-mushrooms-on-blood-cells-under-conditions-of-diabetes-mellitus
#16
REVIEW
Taras Vitak, Borys Yurkiv, Solomon Wasser, Eviatar Nevo, Natalia Sybirna
Diabetes mellitus (DM) is the third most common non-infectious disease leading to early disability and high mortality. Moreover, the number of patients is growing every year. The main symptom of DM is hyperglycemia. Increased levels of blood glucose activate polyol, hexosamine, and protein kinase metabolic pathways cause the intensification of non-enzymatic glycosylation and nitration of macromolecules. This, in turn, leads to the development of oxidative and nitrative stresses and secondary complications, such as different kinds of micro- and macroangiopathies...
May 15, 2017: World Journal of Diabetes
https://www.readbyqxmd.com/read/28536292/capsule-production-and-glucose-metabolism-dictate-fitness-during-serratia-marcescens-bacteremia
#17
Mark T Anderson, Lindsay A Mitchell, Lili Zhao, Harry L T Mobley
Serratia marcescens is an opportunistic pathogen that causes a range of human infections, including bacteremia, keratitis, wound infections, and urinary tract infections. Compared to other members of the Enterobacteriaceae family, the genetic factors that facilitate Serratia proliferation within the mammalian host are less well defined. An in vivo screen of transposon insertion mutants identified 212 S. marcescens fitness genes that contribute to bacterial survival in a murine model of bloodstream infection...
May 23, 2017: MBio
https://www.readbyqxmd.com/read/28528383/pretreatment-of-hardwood-and-miscanthus-with-trametes-versicolor-for-bioenergy-conversion-and-densification-strategies
#18
Ryan M Kalinoski, Hector D Flores, Sunil Thapa, Erin R Tuegel, Michael A Bilek, Evelin Y Reyes-Mendez, Michael J West, Tim J Dumonceaux, Thomas Canam
The pretreatment of plant biomass negatively impacts the economics of many bioenergy and bioproduct processes due to the thermochemical requirements for deconstruction of lignocelluluose. An effective strategy to reduce these severity requirements is to pretreat the biomass with white-rot fungi, such as Trametes versicolor, which have the innate ability to deconstruct lignocellulose with a suite of specialized enzymes. In the present study, the effects of 12 weeks of pretreatment with a wild-type strain (52J) and a cellobiose dehydrogenase-deficient strain (m4D) of T...
May 20, 2017: Applied Biochemistry and Biotechnology
https://www.readbyqxmd.com/read/28498467/antibody-to-human-%C3%AE-fetoprotein-inhibits-cell-growth-of-human-hepatocellular-carcinoma-cells-by-resuscitating-the-pten-molecule-in-vitro-experiments
#19
Kiyoshi Ohkawa, Tadashi Asakura, Yutaka Tsukada, Tomokazu Matsuura
It has been proposed that α-fetoprotein (AFP) is a new member of the intracellular signaling molecule family of the phosphoinositide 3-kinase (PI3K)/AKT signaling pathway via interaction with the phosphatase and tensin homolog (PTEN). In this study, the effects of anti-human AFP antibody on the functions of PTEN were examined using an AFP-producing human hepatoma cell line. The antibody caused significant inhibition of cell growth, compared to a normal IgG control, with the accumulation of intracellular immune complexes followed by significant reduction of cytosolic functional AFP...
May 3, 2017: International Journal of Oncology
https://www.readbyqxmd.com/read/28471544/neonatal-hypothyroidism-affects-testicular-glucose-homeostasis-through-increased-oxidative-stress-in-prepubertal-mice-effects-on-glut3-glut8-and-cx43
#20
D Sarkar, S K Singh
Thyroid hormones (THs) play an important role in maintaining the link between metabolism and reproduction and the altered THs status is associated with induction of oxidative stress in various organs like brain, heart, liver and testis. Further, reactive oxygen species play a pivotal role in regulation of glucose homeostasis in several organs, and glucose utilization by Leydig cells is essential for testosterone biosynthesis and thus is largely dependent on glucose transporter 8 (GLUT8). Glucose uptake by Sertoli cells is mediated through glucose transporter 3 (GLUT3) under the influence of THs to meet energy requirement of developing germ cells...
May 4, 2017: Andrology
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