Askanas V, Serdaroglu P, Engel W K, Alvarez R B
USC Neuromuscular Center, University of Southern California School of Medicine, Los Angeles 90017.
Neurosci Lett. 1991 Sep 2;130(1):73-6. doi: 10.1016/0304-3940(91)90230-q.
In 10/10 inclusion body myositis (IBM) patients and 2/2 oculopharyngeal muscular dystrophy (OPMD) patients, vacuolated muscle fibers contained darkly stained ubiquitin (Ub)-immunoreactive cytoplasmic inclusions. By electronmicroscopy, Ub-immunoreactive material was strictly localized to the 15-21 nm pathologic cytoplasmic tubulofilaments (CTFs). None of 18 control muscle biopsies contained the Ub-immunoreactive inclusions that are typical for IBM and OPMD. Thus, (a) finding that CTFs are ubiquitinated places their protein in the Ub-mediated turnover pathway and provides their first molecular marker; (b) easy accessibility, as compared to the central nervous system, of muscle tissue containing ubiquitinated inclusions should be advantageous for biochemical and molecular studies and may provide information important to both systems.
在10例包涵体肌炎（IBM）患者及2例眼咽型肌营养不良（OPMD）患者中，空泡化肌纤维含有深色的泛素（Ub）免疫反应性胞质内包涵体。通过电子显微镜观察，Ub免疫反应性物质严格定位于15 - 21纳米的病理性胞质微管丝（CTF）。18例对照肌肉活检均未发现IBM和OPMD典型的Ub免疫反应性包涵体。因此，（a）发现CTF被泛素化将其蛋白置于Ub介导的周转途径中，并提供了其首个分子标记；（b）与中枢神经系统相比，含有泛素化包涵体的肌肉组织易于获取，这对生化和分子研究应具有优势，并且可能为两个系统提供重要信息。
