Migheli A, Autilio-Gambetti L, Gambetti P, Mocellini C, Vigliani M C, Schiffer D
2nd Department of Neurology, University of Turin, Italy.
Neurosci Lett. 1990 Jun 22;114(1):5-10. doi: 10.1016/0304-3940(90)90419-a.
The ultrastructural localization of ubiquitin (Ubq) in spinal cord of 2 cases of amyotrophic lateral sclerosis was studied, using immunoperoxidase and immunogold labeling on Vibratome sections or on sections cut from paraffin blocks. Two different types of ubiquitinated cytoplasmic inclusions were observed: (1) bundles composed of 10-15 nm filaments; (2) small rounded bodies without a limiting membrane. A panel of antibodies to neurofilaments (NFs) did not stain the ubiquitinated bundles, and decorated only axonal swellings. Ubq-positive filamentous deposits could be identified with inclusions previously described ultrastructurally. The absence of staining with antibodies to NFs might be due to abnormalities of the filaments, possibly because of a dysfunction of the ubq proteolytic system.
利用免疫过氧化物酶和免疫金标记技术，对振动切片机切片或石蜡块切片进行研究，观察了2例肌萎缩侧索硬化症患者脊髓中泛素（Ubq）的超微结构定位。观察到两种不同类型的泛素化胞质内含物：（1）由10-15nm细丝组成的束状物；（2）无界膜的小圆形体。一组抗神经丝（NFs）抗体未对泛素化束状物染色，仅对轴突肿胀进行了染色。Ubq阳性丝状沉积物可与先前超微结构描述的内含物相鉴别。抗NFs抗体未染色可能是由于细丝异常，可能是由于ubq蛋白水解系统功能障碍所致。
