Espeel M, Roels F, Giros M, Mandel H, Peltier A, Poggi F, Poll-The B T, Smeitink J A, Van Maldergem L, Santos M J
Department of Anatomy, Embryology and Histology, University of Gent, Belgium.
Eur J Cell Biol. 1995 Aug;67(4):319-27.
The presence of peroxisomal membrane ghosts was examined in liver biopsies from eleven patients presenting the clinical and biochemical picture of a generalized peroxisomal disorder (Zellweger syndrome, neonatal adrenoleukodystrophy, infantile Refsum disease and variants of these syndromes). A polyclonal antibody raised against the membrane of human liver peroxisomes and recognizing a 43 kDa peroxisomal membrane protein (PMP) was used. In human control liver the antibodies react in a distinct and specific way with the peroxisomal membrane. Two types of organelles with an immunoreactive membrane were identified in the liver parenchymal cells of the patients: organelles containing an electron-dense core and organelles with electron transparent contents. Both types may co-occur in the same patient; in two patients they were found in the same cell. The organelles are rare, and their number varies between patients. The first type possibly corresponds to the previous morphological description of aberrant peroxisomes in the liver of patients with Zellweger syndrome, neonatal adrenoleukodystrophy and infantile Refsum disease. The empty looking organelles have not been reported previously in the liver, some of the "empty" organelles seem to be enclosed by a double membrane. Morphometrical analysis in three patients indicated that both types of organelles (corrected mean d-circle 0.271-0.306 micron for the "empty" and the dense core organelles, respectively) are smaller than the peroxisomes in postnatal control liver and in fetal liver. In one patient (infantile Refsum disease) immunoreactive organelles were not detected. The organelles with the electron-dense core were not found in two patients.(ABSTRACT TRUNCATED AT 250 WORDS)
在11例呈现全身性过氧化物酶体疾病（泽尔韦格综合征、新生儿肾上腺脑白质营养不良、婴儿型雷夫叙姆病以及这些综合征的变异型）临床和生化表现的患者肝脏活检组织中，检测过氧化物酶体膜空壳的存在情况。使用了一种针对人肝脏过氧化物酶体膜产生的多克隆抗体，该抗体可识别一种43 kDa的过氧化物酶体膜蛋白（PMP）。在人类对照肝脏中，这些抗体与过氧化物酶体膜以独特且特异的方式发生反应。在患者的肝实质细胞中鉴定出两种具有免疫反应性膜的细胞器：含有电子致密核心的细胞器和内容物电子透明的细胞器。这两种类型可能在同一患者中共存；在两名患者中，它们在同一细胞中被发现。这些细胞器很罕见，其数量在不同患者之间有所变化。第一种类型可能与先前对泽尔韦格综合征、新生儿肾上腺脑白质营养不良和婴儿型雷夫叙姆病患者肝脏中异常过氧化物酶体的形态学描述相对应。之前在肝脏中尚未报道过外观呈空泡状的细胞器，一些“空泡状”细胞器似乎被双层膜包围。对三名患者的形态计量分析表明，这两种类型的细胞器（“空泡状”和致密核心细胞器的校正平均d圆直径分别为0.271 - 0.306微米）均小于出生后对照肝脏和胎儿肝脏中的过氧化物酶体。在一名患者（婴儿型雷夫叙姆病）中未检测到免疫反应性细胞器。在两名患者中未发现含有电子致密核心的细胞器。（摘要截断于250字）
