Abstract：
:The purpose of this study was to determine the ultrastructure of the blood-brain barrier (BBB) and blood-spinal cord barrier (BSCB) in G93A SOD1 mice modeling ALS at different stages of disease. Electron microscope examination of brainstem, cervical and lumbar spinal cords was performed in ALS mice at early and late stages of disease. Our results show disorganized mitochondrial cristae and degenerating mitochondria in endothelial cells and neuropil, swollen astrocyte foot processes, swollen and degenerating capillary endothelial cells, astrocytes and motor neurons and extensive extracellular edema. In spite of progressive extracellular edema in neural tissue, capillary endothelial cell tight junctions appeared to remain intact in early and late symptomatic animals. Results show that disruption of BBB and BSCB was evident in areas of motor neuron degeneration in G93A mice at both early and late stages of disease. Capillary rupture was observed in brainstem in early symptomatic G93A mice. Capillary ultrastructure revealed that endothelial cell membrane and/or basement membrane damage occurred, followed by vascular leakage.
journal_name
Brain Resjournal_title
Brain researchauthors
Garbuzova-Davis S,Haller E,Saporta S,Kolomey I,Nicosia SV,Sanberg PRdoi
10.1016/j.brainres.2007.04.044subject
Has Abstractpub_date
2007-07-09 00:00:00pages
126-37eissn
0006-8993issn
1872-6240pii
S0006-8993(07)00972-9journal_volume
1157pub_type
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