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Glycogen storage

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https://www.readbyqxmd.com/read/28542051/critical-airway-stenosis-in-an-adolescent-male-with-pompe-disease-and-thoracic-lordosis-a-case-report
#1
B Randall Brenn, Mary T Theroux, Suken A Shah, William G Mackenzie, Robert Heinle, Mena T Scavina
An adolescent male with late-onset Pompe disease (glycogen storage disease type II) presented with a history of restrictive airway disease and a near-cardiorespiratory arrest during anesthesia for a liver biopsy initially thought to be due to bronchospasm. During a subsequent posterior spinal fusion procedure, he suffered cardiorespiratory arrest resulting in the procedure being aborted. Bronchoscopy performed shortly after resuscitation revealed an undiagnosed narrowing of the distal trachea and bronchi. This is the first description of a patient with lateonset Pompe disease with undiagnosed critical tracheal stenosis due to the progression of thoracic lordosis, which was ultimately relieved by posterior spinal fusion...
May 23, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28541039/label-free-discrimination-of-rhizobial-bacteroids-and-mutants-by-single-cell-raman-microspectroscopy
#2
Jiabao Xu, Isabel Webb, Philip Poole, Wei E Huang
Symbiotic rhizobia in legume account for a large portion of nitrogen fixation in the biosphere. Nitrogen fixation is an energy-demanding process requiring tight control of metabolism and redox state. It is of great interest to understand the bacteroid differentiation process and the roles of energy storage molecules, such as glycogen and polyhydroxybutyrate (PHB), in maintaining the Rhizobium-legume symbioses. Traditional biochemical assays for checking phenotypic changes of mutants require a large volume of starting materials, which is difficult for unculturable, terminally differentiated bacteroids...
May 25, 2017: Analytical Chemistry
https://www.readbyqxmd.com/read/28536182/glucagon-s-effect-on-liver-protein-metabolism-in-vivo
#3
Guillaume Kraft, Katie C Coate, Jason J Winnick, Dominique Dardevet, E Patrick Donahue, Alan D Cherrington, Phillip E Williams, Mary Courtney Moore
The postprandial state is characterized by a storage of nutrients in the liver, muscle and adipose tissue for later utilization. In the case of a protein rich meal, amino acids (AA) stimulate glucagon secretion by the α-cell. The aim of the present study was to determine the impact of the rise in glucagon on AA metabolism, particularly in the liver. We used a conscious catheterized dog model to recreate a postprandial condition using a pancreatic clamp. Portal infusions of glucose, AA and insulin were used to achieve postprandial levels while portal glucagon infusion either maintained the basal level or increased it by 3 fold...
May 23, 2017: American Journal of Physiology. Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28533358/role-of-type-2-nad-p-h-dehydrogenase-ndbc-in-regulation-of-carbon-allocation-in-synechocystis-6803
#4
Tuomas Huokko, Dorota Muth-Pawlak, Natalia Battchikova, Yagut Allahverdiyeva, Eva-Mari Aro
NAD(P)H dehydrogenases comprise type 1 (NDH-1) and type 2 (NDH-2s) enzymes. Even though the NDH-1 complex is a well characterized protein complex in the thylakoid membrane of Synechocystis sp. PCC 6803 (hereafter Synechocystis) the exact roles of different NDH2s remain poorly understood. To elucidate this question we studied the function of NdbC, one of the three NDH-2s in Synechocystis, by constructing a deletion mutant (ΔndbC) for corresponding protein and submitting the mutant to physiological and biochemical characterisation as well as to comprehensive proteomics analysis...
May 22, 2017: Plant Physiology
https://www.readbyqxmd.com/read/28526781/cd36-is-essential-for-endurance-improvement-changes-in%C3%A2-whole-body-metabolism-and-efficient-ppar-related-transcriptional-responses-in-the-muscle-with-exercise-training
#5
Mark Christian C Manio, Shigenobu Matsumura, Daisaku Masuda, Kazuo Inoue
Although circulating fatty acids are utilized as energy substrates, they also function as ligands to the peroxisome-proliferator activated receptors (PPARs), a family of fatty acid sensing transcription factors. Exercise training leads to various adaptations in the muscle such as elevation of glycogen content, mitochondrial number as well as upregulation of fatty acid uptake and utilization through downstream transcriptional adaptations. In line with this, CD36 has been shown to be critical in controlling fatty acid uptake and consequently, fatty acid oxidation...
May 2017: Physiological Reports
https://www.readbyqxmd.com/read/28511025/co-inheritance-of-the-membrane-frizzled-related-protein-ocular-phenotype-and-glycogen-storage-disease-type-ib
#6
Maha Mameesh, Anuradha Ganesh, Beena Harikrishna, Sana Al Zuhaibi, Patrick Scott, Sami Al Kalbani, Khalid Al Thihli
AIM: To report co-occurrence of two rare recessive conditions, the membrane frizzled-related protein (MFRP)-related ocular phenotype and glycogen storage disease type 1b (GSD-1b), in three siblings in an Omani family. BACKGROUND: Biallelic mutations in the MFRP gene (chromosome 11q23) result in a distinct ocular phenotype characterized by retinitis pigmentosa, foveoschisis, optic nerve head drusen, and posterior microphthalmos. GSD-1b is an autosomal-recessive disorder caused by mutations in SLC37A4 gene located in the same chromosomal region...
May 16, 2017: Ophthalmic Genetics
https://www.readbyqxmd.com/read/28502559/3-utr-snp-rs2229611-in-g6pc1-affects-mrna-stability-expression-and-glycogen-storage-disease-type-ia-risk
#7
Sellamuthu Karthi, Mohan Rajeshwari, Amirtharaj Francis, Matheshwaran Saravanan, Perumal Varalakshmi, Henry Houlden, Kumarasamy Thangaraj, Balasubramaniem Ashokkumar
The frequency of rs2229611, previously reported in Chinese, Caucasians, Japanese and Hispanics, was investigated for the first time in Indian ethnicity. We analyzed its role in the progression of Glycogen Storage Disease type-Ia (GSD-Ia) and breast cancer. Genotype data on rs2229611 revealed that the risk of GSD-Ia was higher (P=0.0195) with CC compared to TT/TC genotypes, whereas no such correlation was observed with breast cancer cases. We observed a strong linkage disequilibrium (LD) among rs2229611 and other disease causing G6PC1 variants (|D'|=1, r(2)=1)...
May 11, 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/28497716/polyglucosan-bodies-in-placental-extravillious-trophoblast-for-the-diagnosis-of-fatal-perinatal-neuromuscular-type-glycogen-storage-disease-type-iv
#8
Weiming Yu, Marie-Anne Brundler, James R Wright
The fatal infantile neuromuscular type is the most severe form of glycogen storage disease type IV. We report a case of a 22-day-old female neonate born at 34 weeks gestation with polyhyramnios, fetal hydrops, and severe hypotonia. Placental examination revealed numerous periodic acid schiff (PAS)-positive diastase-resistant polyglucosan bodies in the cytoplasm of extravillous trophoblast predominantly in the placental basal plate. Muscle biopsy and autopsy findings supported a diagnosis of neuromuscular-type glycogen storage disease IV with extensive involvement of skeletal muscle, heart, and liver...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28497340/glycogen-shunt-activity-and-glycolytic-supercompensation-in-astrocytes-may-be-distinctly-mediated-via-the-muscle-form-of-glycogen-phosphorylase
#9
Emil Jakobsen, Lasse K Bak, Anne B Walls, Ann-Kathrin Reuschlein, Arne Schousboe, Helle S Waagepetersen
Glycogen is the main storage form of glucose in the brain. In contrast with previous beliefs, brain glycogen has recently been shown to play important roles in several brain functions. A fraction of metabolized glucose molecules are being shunted through glycogen before reentering the glycolytic pathway, a phenomenon known as the glycogen shunt. The significance of glycogen in astrocyte energetics is underlined by high activity of the glycogen shunt and the finding that inhibition of glycogen degradation, under some conditions leads to a disproportional increase in glycolytic activity, so-called glycolytic supercompensation...
May 11, 2017: Neurochemical Research
https://www.readbyqxmd.com/read/28485502/indole-enhances-the-survival-of-pantoea-ananatis-yj76-in-face-of-starvation-conditions
#10
Jing Zheng, Jiajia Yu, Mengqi Jia, Liping Zheng, Yongjun Feng
Pantoea ananatis YJ76 is an indole-producing predominant diazotrophic endophyte isolated from rice having multiple growth-promoting effects on host plant. As a decomposition metabolite of L-tryptophan (L-Trp), indole is confirmed to regulate various physiological processes of bacteria. In this research, we found that indole significantly improves the survival of YJ76 in face of starvation conditions and the promoting effect is related to the glycogen accumulation promoted by indole, which is much more significant in the middle decline phase than in other growth phases...
May 9, 2017: Journal of Basic Microbiology
https://www.readbyqxmd.com/read/28483667/effect-of-brain-derived-neurotrophic-factor-bdnf-on-hepatocyte-metabolism
#11
Yoni Genzer, Nava Chapnik, Oren Froy
Brain-derived neurotrophic factor (BDNF) plays crucial roles in the development, maintenance, plasticity and homeostasis of the central and peripheral nervous systems. Perturbing BDNF signaling in mouse brain results in hyperphagia, obesity, hyperinsulinemia and hyperglycemia. Currently, little is known whether BDNF affects liver tissue directly. Our aim was to determine the metabolic signaling pathways activated after BDNF treatment in hepatocytes. Unlike its effect in the brain, BDNF did not lead to activation of the liver AKT pathway...
May 5, 2017: International Journal of Biochemistry & Cell Biology
https://www.readbyqxmd.com/read/28482395/-one-case-of-x-linked-recessive-glycogen-storage-disease-type-ixa
#12
H M Guo, B X Zheng, M Li
No abstract text is available yet for this article.
May 4, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28475761/sugar-versus-fat-elimination-of-glycogen-storage-improves-lipid-accumulation-in-yarrowia-lipolytica
#13
Govindprasad Bhutada, Martin Kavšcek, Rodrigo Ledesma-Amaro, Stéphane Thomas, Gerald N Rechberger, Jean-Marc Nicaud, Klaus Natter
Triacylglycerol (TAG) and glycogen are the two major metabolites for carbon storage in most eukaryotic organisms. We investigated the glycogen metabolism of the oleaginous Yarrowia lipolytica and found that this yeast accumulates up to 16% glycogen in its biomass. Assuming that elimination of glycogen synthesis would result in an improvement of lipid accumulation, we characterized and deleted the single gene coding for glycogen synthase, YlGSY1. The mutant was grown under lipogenic conditions with glucose and glycerol as substrates and we obtained up to 60% improvement in TAG accumulation compared to the wild-type strain...
May 1, 2017: FEMS Yeast Research
https://www.readbyqxmd.com/read/28473467/hepatic-protein-phosphatase-1-regulatory-subunit-3b-ppp1r3b-promotes-hepatic-glycogen-synthesis-and-thereby-regulates-fasting-energy-homeostasis
#14
Minal B Mehta, Swapnil V Shewale, Raymond N Sequeira, John S Millar, Nicholas J Hand, Daniel J Rader
Maintenance of whole-body glucose homeostasis is critical to glycemic function. Genetic variants mapping to chromosome 8p23.1 in genome-wide association studies have been linked to glycemic traits in humans. The gene of known function closest to the mapped region, PPP1R3B (protein phosphatase 1 regulatory subunit 3B), encodes a protein (GL) that regulates glycogen metabolism in the liver. We therefore sought to test the hypothesis that hepatic PPP1R3B is associated with glycemic traits. We generated mice with either liver-specific deletion (Ppp1r3bΔhep) or liver-specific overexpression of Ppp1r3b...
May 4, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28465215/glycogen-synthase-from-the-parabasalian-parasite-trichomonas-vaginalis-an-unusual-member-of-the-starch-glycogen-synthase-family
#15
Wayne A Wilson, Prajakta Pradhan, Nayasha Madhan, Galen C Gist, Andrew Brittingham
Trichomonas vaginalis, a parasitic protist, is the causative agent of the common sexually-transmitted infection trichomoniasis. The organism has long been known to synthesize substantial glycogen as a storage polysaccharide, presumably mobilizing this compound during periods of carbohydrate limitation, such as might be encountered during transmission between hosts. However, little is known regarding the enzymes of glycogen metabolism in T. vaginalis. We had previously described the identification and characterization of two forms of glycogen phosphorylase in the organism...
April 29, 2017: Biochimie
https://www.readbyqxmd.com/read/28461393/crebh-maintains-circadian-glucose-homeostasis-by-regulating-hepatic-glycogenolysis-and-gluconeogenesis
#16
Hyunbae Kim, Ze Zheng, Paul D Walker, Gregory Kapatos, Kezhong Zhang
Cyclic AMP responsive element binding protein, hepatic specific (CREBH) is a liver-enriched, endoplasmic reticulum-tethered transcription factor known to regulate hepatic acute-phase response and lipid homeostasis. In this study, we demonstrated that CREBH functions as a circadian transcriptional regulator that plays major roles in maintaining glucose homeostasis. CREBH proteolytic cleavage and post-translational acetylation modification are regulated under the circadian clock. Functionally, CREBH is required to maintain circadian homeostasis of hepatic glycogen storage and blood glucose levels...
May 1, 2017: Molecular and Cellular Biology
https://www.readbyqxmd.com/read/28458779/genetic-disorders-in-beef-cattle-a-review
#17
REVIEW
Aleksandra Ciepłoch, Karolina Rutkowska, Jolanta Oprządek, Ewa Poławska
The main purpose of present review is to describe and organize autosomal recessive disorders (arachnomelia, syndactylism, osteopetrosis, dwarfism, crooked tail syndrome, muscular hyperplasia, glycogen storage disease, protoporphyria), which occur among beef cattle, and methods that can be applied to detect these defects. Prevalence of adverse alleles in beef breeds happens due to human activity-selections of favorable features, e.g. developed muscle tissue. Unfortunately, carriers of autosomal recessive diseases are often characterized by these attributes...
2017: Genes & Genomics
https://www.readbyqxmd.com/read/28458274/supplementation-with-hualian-no-4-wild-bitter-gourd-momordica-charantia-linn-var-abbreviata-ser-extract-increases-anti-fatigue-activities-and-enhances-exercise-performance-in-mice
#18
Chien-Yu Hsiao, Yi-Ming Chen, Yi-Ju Hsu, Chi-Chang Huang, Hsin-Ching Sung, Sheng-Shih Chen
Hualian No. 4 wild bitter gourd (WBG) is a specific vegetable cultivated by the Hualien District Agricultural Research and Extension Station in Taiwan. WBG is commonly consumed as a vegetable and used as a popular folk medicine. However, few studies have demonstrated the effects of WBG supplementation on exercise performance, physical fatigue and the biochemical profile. The purpose of this study was to evaluate the potential beneficial effects of WBG extract on fatigue and ergogenic functions following physiological challenge...
May 1, 2017: Journal of Veterinary Medical Science
https://www.readbyqxmd.com/read/28453664/glycogen-synthesis-in-glycogenin-1-deficient-patients-a-role-for-glycogenin-2-in-muscle
#19
Thomas O Krag, Cristina Ruiz Ruiz, John Vissing
Context: Glycogen storage disease type XV (GSD XV) is a rare disease caused by mutations in the GYG1 gene that codes for the core molecule of muscle glycogen, glycogenin 1. Nonetheless, glycogen is present in muscles of glycogenin 1 deficient patients, suggesting an alternative for glycogen build-up. A likely candidate is glycogenin 2, an isoform expressed in liver and heart, but not in healthy skeletal muscle. Objective: We wanted to investigate the formation of glycogen and changes in glycogen metabolism in patients with GSD XV...
April 27, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28453529/effect-of-mild-hypothermia-preconditioning-against-low-temperature-4%C3%A2-c-induced-rat-liver-cell-injury-in-vitro
#20
Jiasheng Qin, Yanxing Mai, Yang Li, Zesheng Jiang, Yi Gao
Bioartificial liver holds special position in the field of regenerative medicine, and cold environment at 4℃ is widely used for the short storage of both organ and liver cell for later application. However, the disadvantages of such cold storage could influence cell viability and lead to cell apoptosis in different degrees. In this study, we mainly explore the pre-protective effect of mild hypothermia against low temperature (4℃)-induced rat liver cell injury in vitro. Our results indicated that the precondition with mild hypothermia could increase cell viability, such as cell proliferation, LDH regulation and glycogen synthesis ability of liver cell...
2017: PloS One
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