Abstract:
:Selective degeneration and death of motor neurons in SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS) is accompanied by axonal disorganization and reduced slow axonal transport in the three most frequently used mouse models of mutant SOD1-mediated ALS. To test whether suppression of axonal degeneration (frequently known as Wallerian degeneration) could slow disease development, we took advantage of a spontaneous mouse mutant Wld(s) (Wallerian degeneration slow) in which the programmed axonal degenerative process that is normally activated after axonal injury is significantly delayed. Despite its effectiveness in delaying axonal loss in other neurodegenerative models, the presence of Wld(s) did not slow disease onset, ameliorate mutant motor neuron death, axonal degeneration, or preserve synaptic attachments in mice that develop disease from ALS-linked SOD1 mutants SOD1G37R or SOD1G85R. However, presynaptic endings in both the presence and absence of Wld(s) showed high accumulations of mitochondria and synaptic vesicles, implicating errors of retrograde transport as a consequence of SOD1-mutant damage to axons.
journal_name
Neuromolecular Medjournal_title
Neuromolecular medicineauthors
Vande Velde C,Garcia ML,Yin X,Trapp BD,Cleveland DWdoi
10.1385/NMM:5:3:193keywords:
subject
Has Abstractpub_date
2004-01-01 00:00:00pages
193-203issue
3eissn
1535-1084issn
1559-1174pii
NMM:5:3:193journal_volume
5pub_type
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