Wiggins Larisa M
Department of Physiology and Cell Biology, University of Nevada, Reno.
Oxid Antioxid Med Sci. 2014;3(3):161-173. doi: 10.5455/oams.310714.or.071.
There is increased evidence that oxidative stress is involved in exacerbations of neurodegenerative diseases and spinal muscular atrophies.
We examined changes in morphology and expression of antioxidant proteins and peroxiredoxins in motor neurons of lumbar spinal cord, dorsal root ganglion sensory neurons, macroglial cells and quadriceps muscles of newborn heterozygous Loa/+ mice ("legs at odd angles"), a mouse model for early onset of the spinal muscular atrophy with lower extremity predominance (SMA-LED).
Our data indicate that newborn Loa-mice develop: neuroinflammation of the sensory and motor neurons; muscular inflammation with atrophic and denervated myofibers; increased expression of neuronal mitochondrial peroxiredoxins (Prxs) 3, 5 and cytoplasmic Prx 6 in motor and sensory neurons, myofibers, fibroblasts of perimysium and chondrocytes of cartilage; and decreased expression of Prx 6 by glial cells and in extracellular space surrounding motor neurons.
The decrease in expression of Prx 6 by glial cells and extracellular Prx 6 secretion in early stages of the pathological conditions is consistent with the hypothesis that chronic oxidative stress may lead to neurodegeneration of motor neurons and exacerbation of the pathology.
越来越多的证据表明氧化应激参与神经退行性疾病和脊髓性肌萎缩症的病情加重过程。
我们检测了新生杂合Loa/+小鼠（“腿部呈奇怪角度”）的腰脊髓运动神经元、背根神经节感觉神经元、大胶质细胞和股四头肌中抗氧化蛋白和过氧化物酶的形态变化及表达情况，该小鼠模型为下肢为主型脊髓性肌萎缩症（SMA-LED）的早发型。
我们的数据表明新生Loa小鼠出现：感觉和运动神经元的神经炎症；伴有萎缩和失神经支配肌纤维的肌肉炎症；运动和感觉神经元、肌纤维、肌束膜成纤维细胞和软骨细胞中神经元线粒体过氧化物酶（Prxs）3、5和细胞质Prx 6的表达增加；神经胶质细胞以及运动神经元周围细胞外空间中Prx 6的表达降低。
在病理状况早期神经胶质细胞中Prx 6表达的降低以及细胞外Prx 6的分泌，与慢性氧化应激可能导致运动神经元神经变性和病情加重这一假说相符。
