Morphologic Features of the Liver in Abetalipoproteinemia
Morphologic Features of the Liver in Abetalipoproteinemia
复制标题
无β脂蛋白血症肝脏的形态特征
DOI：
10.1002/hep.1840040621
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发表时间：
1984
期刊：
影响因子：
13.5
通讯作者：
J. Hoofnagle
中科院分区：
文献类型：
--
作者：
M. Avigan;K. Ishak;R. Gregg;J. Hoofnagle
本文用光镜和电镜研究了一例新诊断的30岁乙型蛋白血症患者的肝脏组织。尽管肝细胞内积聚了大量脂滴，但小叶结构保持完整，未发生纤维化。棘细胞在扫描电镜下很容易被识别。透射电镜下粗面内质网、光滑面内质网及高尔基体形态正常。脂滴不与膜结合，也不与内质网或高尔基体相关。在低脂蛋白血症中，这些形态特征与载脂蛋白B合成缺陷一致。这阻止了脂蛋白的组装，而脂蛋白可以通过正常的细胞内通讯小管网络分泌，因此，脂肪在肝细胞的细胞质中积累。
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.
影响因子：
15.9
作者：
MALLOY, MJ;KANE, JP;DALAL, KB
通讯作者：
DALAL, KB
载脂蛋白在人肠道中的定位和定量。
影响因子：
29.4
作者：
Green,PH;Lefkowitch,JH;Glickman,RM;Riley,JW;Quinet,E;Blum,CB
通讯作者：
Blum,CB
载脂蛋白 B 在人空肠吸收细胞内的超微结构免疫定位。
影响因子：
6.5
作者：
Christensen,NJ;Rubin,CE;Cheung,MC;Albers,JJ
通讯作者：
Albers,JJ