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Morphologic Features of the Liver in Abetalipoproteinemia
Author(s) -
Avigan Mark I.,
Ishak Kamal G.,
Gregg Richard E.,
Hoofnagle Jay H.
Publication year - 1984
Publication title -
hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 5.488
H-Index - 361
eISSN - 1527-3350
pISSN - 0270-9139
DOI - 10.1002/hep.1840040621
Subject(s) - endoplasmic reticulum , golgi apparatus , lipid droplet , cytoplasm , golgi membrane , electron microscope , transmission electron microscopy , biology , intracellular , microbiology and biotechnology , chemistry , pathology , biochemistry , materials science , nanotechnology , medicine , physics , optics
Liver tissue of a newly diagnosed 30‐year‐old patient with abetalipoproteinemia was studied by light and electron microscopy. Despite accumulation of large quantities of lipid droplets in hepatocytes, the lobular architecture remained intact, and there was no fibrosis. Acanthocytes were readily identified in sinusoids by scanning electron microscopy. Profiles of rough endoplasmic reticulum, smooth endoplasmic reticulum and Golgi apparatus appeared normal by transmission electron microscopy. Lipid droplets were not bound to membranes and were not associated with the endoplasmic reticulum or Golgi apparatus. In abetalipoproteinemia, these morphological features are consistent with defective apolipoprotein B synthesis. This prevents assembly of lipoproteins that can be secreted through the normal intracellular communicating tubular network and, consequently, fat accumulates in the cytoplasm of the hepatocytes.