Stereological analysis of peroxisomes and mitochondria in intestinal epithelium of patients with peroxisomal deficiency disorders: Zellweger's syndrome and neonatal-onset adrenoleukodystrophy.
Stereological analysis of peroxisomes and mitochondria in intestinal epithelium of patients with peroxisomal deficiency disorders: Zellweger's syndrome and neonatal-onset adrenoleukodystrophy.
复制标题
过氧化物酶体缺乏症患者肠上皮中过氧化物酶体和线粒体的体视学分析：齐薇格综合征和新生儿发病的肾上腺脑白质营养不良。
DOI：
10.1002/aja.1001770112
复制
发表时间：
1986
期刊：
影响因子：
--
通讯作者：
Cornacchia3rd,L
中科院分区：
文献类型：
--
作者：
Black,VH;Cornacchia3rd,L
参与脂质代谢的过氧化物酶体，已被证明在两种代谢疾病（长链脂肪酸在组织中积聚）的肝脏中发生改变：齐薇格综合征和新生儿肾上腺白质营养不良（ALD）。肠道也在脂质代谢中发挥作用，我们利用细胞化学和体视学分析相结合的技术，在电镜水平上比较了正常肠上皮与齐薇格综合征和新生儿ALD患者的过氧化物酶体。正常人肠上皮中有大量过氧化物酶体。它们呈椭球状，平均直径为0.37 × 0.56 μm，填充有粗颗粒状的DAB +。ALD患者肠上皮内的过氧化物酶体外观和数量相似，但尺寸较小（0.28 × 0.44 μm）。齐薇格综合征患者肠上皮中缺乏外观正常的过氧化物酶体；然而，在小得多的尺寸（0.12 × 0.19 μm）的膜结合结构中观察到DAB +的含量。齐薇格综合征患者的肝脏缺乏过氧化物酶体；在新生儿ALD中，它们在外观上异常且数量大大减少。齐韦格综合征患者肠上皮中存在罕见的微小过氧化物酶体，新生儿ALD患者肠上皮中存在小过氧化物酶体，表明这些疾病中肠上皮中的过氧化物酶体受到影响，但影响程度低于肝脏。在ALD肠上皮中，DAB +物质也见于长状、弯曲状、管状或池状成分，与过氧化物酶体混合，偶尔与过氧化物酶体连续。有人认为，这些代表了过氧化物酶体形成的早期阶段，即Lazarow最初设想的过氧化物酶体网状结构，而Zellweger所看到的罕见结构代表了这种网状结构的雏形。在这些结构附近的细胞质中出现了片层内含物和清晰的空间，表明在固定过程中积累的物质被提取出来，或者这些区域更容易发生自溶。线粒体也参与脂质代谢，在齐薇格氏组织中有异常的报道。本研究未观察到肠上皮线粒体的质的差异。虽然定量显示新生儿ALD肠上皮线粒体的平均体积、数量和表面密度更大，但在所有病例中并无统计学差异。
Peroxisomes, participants in lipid metabolism, have been shown to be altered in liver in two metabolic diseases in which long‐chain fatty acids accumulate in tissues: Zellweger's syndrome and neonatal adrenoleukodystrophy (ALD). The intestine also plays a role in lipid metabolism, and we have had the opportunity to compare peroxisomes in normal intestinal epithelium with those from patients with Zellweger's syndrome and neonatal ALD at the electron microscopic level by using the combined techniques of cytochemistry and stereological analysis. Peroxisomes were numerous in intestinal epithelium of the normal individuals. They were ellipsoidal in shape with average diameters of 0.37 by 0.56 μm and filled with coarsely granular, DAB + content. Peroxisomes in the intestinal epithelium of the ALD patient were similar in appearance and number but smaller in size (0.28 by 0.44 μm). Peroxisomes of normal appearance were absent from the intestinal epithelium of patients with Zellweger's syndrome; DAB + content, however, was observed in rare, membrane‐bound structures of much smaller size (0.12 by 0.19 μm). In liver of patients with Zellweger's syndrome, peroxisomes are lacking; in neonatal ALD they are abnormal in appearance and greatly reduced in number. The presence of rare minute peroxisomes in the intestinal epithelium in Zellweger's syndrome and of small peroxisomes in this epithelium in neonatal ALD indicate that peroxisomes in the intestinal epithelium are affected in these diseases, but to a lesser extent than in the liver. In the ALD intestinal epithelium, DAB + material was also seen in long, sinuous, tubular or cisternal elements intermingled and occasionally in continuity with peroxisomes. It is suggested that these represent the early stages of peroxisome formation, the peroxisomal reticulum as originally envisioned by Lazarow, while the rare structures seen in Zellweger's represent rudiments of such a reticulum. Lamellar inclusions and clear spaces occurred in the cytoplasm adjacent to these structures indicating either that material accumulated there had been extracted during fixation or that these regions are more susceptible to autolysis. Mitochondria are also involved in lipid metabolism and have been reported to be abnormal in Zellweger's tissue. No qualitative differences were observed in the mitochondria of the intestinal epithelia examined in this study. Although quantitation revealed a greater mean volume, number, and surface density of mitochondria in the intestinal epithelia of neonatal ALD, it was not a statistically significant difference in all cases.