Results 21 to 30 of about 1,002,705 (170)
Liver Transplantation for Glycogen Storage Disease Type IV
Frontiers in Pediatrics, 2021 Glycogen storage disease type IV (GSD IV) is a rare autosomal recessive disorder caused by glycogen–branching enzyme (GBE) deficiency, leading to accumulation of amylopectin–like glycogen that may damage affected tissues.
Min Liu +4 more
doaj +1 more source
The influence of amylose content on the modification of starches by glycogen branching enzymes
Food Chemistry, 2022 Glycogen branching enzymes (GBEs) have been used to generate new branches in starches for producing slowly digestible starches. The aim of this study was to expand the knowledge about the mode of action of these enzymes by identifying structural aspects of starchy substrates affecting the products generated by different GBEs.
Gaenssle, Aline L O +2 more
openaire +3 more sources
Frontiers in Neurology, 2023 Inadequate glycogen branching enzyme 1 (GBE1) activity results in different forms of glycogen storage disease type IV, including adult polyglucosan body disorder (APBD).
Joseph R. Abraham +5 more
doaj +1 more source
Molecular Genetics and Metabolism Reports, 2018 Background: Glycogen storage disease type IV (GSD IV), caused by GBE1 mutations, has a quite wide phenotypic variation. While the classic hepatic form and the perinatal/neonatal neuromuscular forms result in early mortality, milder manifestations include
Hiroyuki Iijima +7 more
doaj +1 more source
Hypoxia promotes glycogen accumulation through hypoxia inducible factor (HIF)-mediated induction of glycogen synthase 1. [PDF]
PLoS ONE, 2010 When oxygen becomes limiting, cells reduce mitochondrial respiration and increase ATP production through anaerobic fermentation of glucose. The Hypoxia Inducible Factors (HIFs) play a key role in this metabolic shift by regulating the transcription of ...
Nuria Pescador +12 more
doaj +1 more source
Children, 2023 Glycogen storage disease type IV (GSD IV) (OMIM #232500) is an autosomal recessive disorder caused by deficiency of the glycogen-branching enzyme. Here, we report a patient presenting with prematurity and severe hypotonia resulting from a complicated ...
Handan Bezirganoglu, Kubra Adanur Saglam
doaj +1 more source
The versatile activity of glycogen branching enzymes [PDF]
, 2020 Glycogen branching enzymes (GBEs) are active in the biosynthesis of glycogen and can be applied to modify starch. These enzymes have been described to introduce new branch points but their exact mechanism is still little understood due to the size and complexity of both their substrate and product.
openaire +2 more sources
Glycogen synthase isoforms in Synechocystis sp. PCC6803: identification of different roles to produce glycogen by targeted mutagenesis. [PDF]
PLoS ONE, 2014 Synechocystis sp. PCC6803 belongs to cyanobacteria which carry out photosynthesis and has recently become of interest due to the evolutionary link between bacteria and plant species.
Sang-Ho Yoo +4 more
doaj +1 more source
Starch branching enzymes and their genes in Sorghum [PDF]
, 2004 Starch is an important raw material both for food and non-food purposes. It is synthesized and stored in source and sink tissues in plants. The starch deposited in amyloplasts of storage tissues possesses several physico-chemical properties, which makes ...
Mutisya, Joel
core +1 more source
Frontiers in Microbiology, 2021 Glycogen is a polysaccharide that comprises α-1,4-linked glucose backbone and α-1,6-linked glucose polymers at the branching points. It is widely found in organisms ranging from bacteria to eukaryotes.
Areum Lee +5 more
doaj +1 more source