Results 1 to 10 of about 3,807 (135)

The Synergistic Effect of GH13 and GH57 GBEs of Petrotoga mobilis Results in α-Glucan Molecules with a Higher Branch Density. [PDF]

Polymers (Basel), 2023
Glycogen is a biopolymer consisting of glycosyl units, with a linear backbone connected by α-1,4-linkages and branches attached via α-1,6-linkages. In microorganisms, glycogen synthesis involves multiple enzymes, with glycogen branching enzymes (GBEs ...
Bax HHM, Gaenssle AL, van der Maarel MJEC, Jurak E.   +3 more
europepmc   +7 more sources

Diverse electrophysiological demyelinating features in a late-onset glycogen storage disease type IIIa case [PDF]

Open Medicine
Glycogen storage disease type IIIa (GSD IIIa) is a rare etiology among patients with adult-onset myopathy, which is typically associated with axonopathy rather than demyelination.
Zhou Xiajun, Zhong Xingxing, Gao Mingshi, Yue Dongyue, Qiao Kai, Wang Min, Zhi Nan, Cao Wenwei, Han Lu, Lu Jiahong, Zhu Wenhua, Zhao Chongbo, Guan Yangtai   +12 more
doaj   +2 more sources

Generation of a GDE heterozygous mutation human embryonic stem cell line WAe001-A-14 by CRISPR/Cas9 editing

Stem Cell Research, 2018
Glycogen debranching enzyme (GDE) plays a critical role in glycogenolysis. Mutations in the GDE gene are associated with a metabolic disease known as glycogen storage disease type III (GSDIII).
Guosheng Xu, Dongsheng Guo, Feima Wu, Nasir Abbas, Keyu Lai, Fang Yuan, Kai You, Yanli Liu, Yuanqi Zhuang, Yuhang Wu, Yingying Xu, Yan Chen, Fan Yang, Tingcai Pan, Yin-xiong Li   +14 more
doaj   +1 more source

Molecular and clinical characterization of Colombian patients suffering from type III glycogen storage disease

Biomédica: revista del Instituto Nacional de Salud, 2018
Introduction: Type III glycogen storage disease (GSD III) is an autosomal recessive disorder in which a mutation in the AGL gene causes deficiency of the glycogen debranching enzyme.
Carolina Mantilla, Mónica Toro, María Elsy Sepúlveda, Margarita Insuasty, Diana di Filippo, Juan Álvaro López, Carolina Baquero, María Cristina Navas, Andrés Augusto Arias   +8 more
doaj   +1 more source

An extracellular cell-attached pullulanase confers branched α-glucan utilization in human gut Lactobacillus acidophilus [PDF]

, 2017
Of the few predicted extracellular glycan-active enzymes, glycoside hydrolase family 13 subfamily 14 (GH13_14) pullulanases are the most common in human gut lactobacilli.
Abou Hachem, Maher, Celebioglu, Hasan Ufuk, Cypryk, Wojciech, Goh, Yong Jun, Klaenhammer, Todd R, Møller, Marie Sofie, Rasmussen, Kasper Bøwig, Svensson, Birte   +7 more
core   +1 more source

Glycogen and its metabolism: some new developments and old themes [PDF]

, 2012
Glycogen is a branched polymer of glucose that acts as a store of energy in times of nutritional sufficiency for utilization in times of need. Its metabolism has been the subject of extensive investigation and much is known about its regulation by ...
Depaoli-Roach, Anna A., Hurley, Thomas D., Roach, Peter J., Tagliabracci, Vincent S.   +3 more
core   +1 more source

Liver transplantation for type I and type IV glycogen storage disease [PDF]

, 1993
Progressive liver failure or hepatic complications of the primary disease led to orthotopic liver transplantation in eight children with glycogen storage disease over a 9-year period.
A. G. Tzakis, AS Guerra, B Illingworth, B. I. Brown, BI Brown, BI Brown, BI Brown, CA Alper, D Raum, DA Merrill, E. Yunis, GA Bannayan, GB Reed, GM Greene, GT Cori, H Zellweger, HG Hers, HL Greene, IT Ferguson, JJ Malatack, JJ Siekierka, KR McMaster, MW Harding, N Kashiwagi, N Kashiwagi, R. S. Kendall, R. Selby, RC Nordlie, S Servidei, S. Todo, T Ishihara, T. E. Starzl, TE Starl, TE Starzl, TE Starzl, TE Starzl, TE Starzl, V Mazzaferro   +37 more
core   +1 more source

On the kinetic and allosteric regulatory properties of the ADP-glucose pyrophosphorylase from Rhodococcus jostii: An approach to evaluate glycogen metabolism in oleaginous bacteria [PDF]

, 2016
Rhodococcus spp. are oleaginous bacteria that accumulate glycogen during exponential growth. Despite the importance of these microorganisms in biotechnology, little is known about the regulation of carbon and energy storage, mainly the relationship ...
Alvarez, Hector Manuel, Asención Diez, Matías Damián, Cereijo, Antonela Estefanía, Dávila Costa, José Sebastián, Iglesias, Alberto Alvaro   +4 more
core   +1 more source

Lafora disease offers a unique window into neuronal glycogen metabolism [PDF]

, 2018
Lafora disease (LD) is a fatal, autosomal recessive, glycogen-storage disorder that manifests as severe epilepsy. LD results from mutations in the gene encoding either the glycogen phosphatase laforin or the E3 ubiquitin ligase malin. Individuals with LD
Gentry, Matthew S., Guinovart, Joan J., Minassian, Berge A., Roach, Peter J., Serratosa, Jose M.   +4 more
core   +2 more sources

Technical and Comparative Aspects of Brain Glycogen Metabolism. [PDF]

, 2019
It has been known for over 50 years that brain has significant glycogen stores, but the physiological function of this energy reserve remains uncertain.
A Nakayama, AB Walls, AF Soares, AM Brown, AM Brown, AM Cataldo, C Cali, C Smythe, CH Phelps, CJ Cummins, CL Poitry-Yamate, D Bornstadt von, D Feuerstein, D Stapleton, DA MacLean, DG Allen, DJ Baker, DW McCandless, E Goldsmith, E Malucelli, E Melendez-Hevia, EN Genda, G Oz, G Testoni, GA Dienel, GA Dienel, GA Dienel, H Watanabe, I Marchand, I Saez, IA Silver, IY Choi, J Cammermeyer, J Folbergrova, J Koizumi, J Kong, J Nielsen, J Vissing, JH Youn, JL Seidel, JM Pivarnik, K Sahlin, KV Subbarao, KV Subbarao, L Pellerin, L Sokoloff, LA Cavalcante, LP Garetto, M Cambray-Deakin, M Cambray-Deakin, M DiNuzzo, M DiNuzzo, M Haggstrom, M Ingvar, M Kusunoki, M Nannipieri, MA Kai Kai, MA Medina, MF Thompson, MZ Ibrahim, N Ortenblad, NF Cruz, O Sorg, OH Lowry, P Elsner, PC Calder, PJ Magistretti, PJ Magistretti, R Dringen, R Geddes, R Gruetter, R Wender, RA Ratcheson, RA Swanson, RA Swanson, RA Swanson, RA Swanson, RA Swanson, RC Borke, RG Shulman, RG Shulman, RH Pudenz, RK Conlee, RL Khandelwal, RR Cardell Jr, S Jiang, S Suda, SB Tekkok, SM Sagar, SR Nelson, SW Suh, SW Suh, T Nelson, U Ponten, Y Cao, Y Oe, Z Gunja-Smith   +96 more
core   +1 more source