Figure 5 - available via license: Creative Commons Attribution 4.0 International
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Crystal structures of the homodimeric human glycogenin-1 complexed with UDP and maltotetraose (PDB 3U2U; the binding regions of ligands are highlighted by ovals) and the apo protein (PDB 3Q4S; the interior empty space of the protein is indicated by red dots).
Source publication
Seventeen out of 764 liver biopsies from transplanted (Tx) livers in children showed glycogen-ground glass (GGG) hepatocytic inclusions. The inclusions were not present in pre-Tx or in the explanted or donor’s liver. Under the electron microscope (EM), the stored material within the cytosol appeared as non-membrane-bound aggregates of electron-luce...
Context in source publication
Context 1
... carried out the docking search within 30 Å of the centre of the protein empty space determined with Hollow [29], which also contains the binding pockets of the natural ligands of glycogenin, uridine-5 -diphosphate (UDP) and sugars ( Figure 5). Binding affinities for the docked drug-glycogenin complexes were calculated with Cyscore [30]. ...
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Citations
... On electron microscopy, these inclusions are composed of non-membrane-bound collections of abnormal glycogen along with some degenerating cell organelles [56][57][58]. They are also associated with several glycogen-associated proteins such as glycogenin-1, and it is thought that these inclusions result from disturbed glycogen metabolism induced by drugs binding to glycogenin [59]. Drugs may also result in hepatocytes having a "two-tone" cytoplasmic appearance that is highlighted by PAS stain ( Figure 6B); in this histological pattern, the hepatocytes show normal cytoplasmic eosinophilia in one-half and a distinctive homogeneous gray appearance in the other half [60]. ...
... Pseudoground glass changes refer to large amphop to-pale eosinophilic inclusion-like structures within hepatocytes that are single, positive, diastase sensitive, and often surrounded by a rim of normal cytoplasm (F 6A). On electron microscopy, these inclusions are composed of non-membrane-b collections of abnormal glycogen along with some degenerating cell organelles [56 They are also associated with several glycogen-associated proteins such as glycogen and it is thought that these inclusions result from disturbed glycogen metabolism ind by drugs binding to glycogenin [59]. Drugs may also result in hepatocytes having a " tone" cytoplasmic appearance that is highlighted by PAS stain ( Figure 6B); in histological pattern, the hepatocytes show normal cytoplasmic eosinophilia in one and a distinctive homogeneous gray appearance in the other half [60]. ...
The liver is a major store of glycogen and is essential in maintaining systemic glucose homeostasis. In healthy individuals, glycogen synthesis and breakdown in the liver are tightly regulated. Abnormal glycogen metabolism results in prominent pathological changes in the liver, often manifesting as hepatic glycogenosis or glycogen inclusions. This can occur in genetic glycogen storage disease or acquired conditions with insulin dysregulation such as diabetes mellitus and non-alcoholic fatty liver disease or medication effects. Some primary hepatic tumors such as clear cell hepatocellular carcinoma also demonstrate excessive glycogen accumulation. This review provides an overview of the pathological manifestations and molecular mechanisms of liver diseases associated with abnormal glycogen accumulation.
