terminal portion of the protein, a pleckstrin homology domain and a DBL homology domain have been identified. A mutation found in one of the families that spares the shorter polypeptide but eliminates the full-length protein resulted in a distinct form of motor neuron disease termedprimary lateral...
Cys6 and Cys111 are in the free thiol state, while Cys57 and Cys146 form an intramolecular disulfide bond. Full size image SOD1 has been proposed to exhibit toxic properties upon forming large aggregates such as fibrils and small oligomers such as trimers5,6,15,16. Insoluble large ...
NSCsWT were prepared from transgenic mice expressing the full length of human wild-type SOD1. NSCs isolated from non-transgenic mice (NSC−/−) were used as controls. The cells were maintained in complete StemPro NSC media to form neurospheres and then subcultured at a 1:3 ratio every ...
Download: Download full-size image Figure 5. SOD1 and Ubiquitin Immunoreactive Inclusions in Neuronal Cell Bodies and Processes in G85R Transgenic Mice Are Early Hallmarks of Disease That Increase in Abundance at End-Stage Disease (A and B) Early neuronal abnormalities in G85R transgenic animals...
Full-length images of Western blots are presented in Supplemental Fig. S6. spinal cords were determined by normalizing the amount of Western blots (Fig. 6b) to the amount of that same protein +prNesHen2Ot HonbtohuencdoprrreostpeionnddeintegctiendpuotnWtheestaecrynl-bRlAotCs (Fig...
A literature search of publications pertaining to SOD1-ALS and its treatment from 1992-present using the MEDLINE database form the basis for this review. Expert opinion: Central nervous system SOD1 knockdown is achievable in SOD1-ALS patients with intrathecal antisense oligonucleotide therapy, and ...
Full size image Figure 2 Native mass spectra of noncovalent complexes of SOD1 and EGCG at molar ratio 1:6 of SOD1:ligand (a), IMS-MS driftscope plots of SOD1 and SOD1-EGCG complex total species (b), the extracted mass spectra of the dimeric SOD1 and SOD1-EGCG complex species from...
Full size image PDI is S-nitrosylated in astrocytes following OGD/reperfusion; this S-nitrosylation of PDI is blocked by iNOS inhibitor 1400W We investigated whether or not aberrant generation of NO through activation of iNOS mediated S-nitrosylation of PDI in reactive astrocytes following OGD/reperf...
Full size image PDI is S-nitrosylated in astrocytes following OGD/reperfusion; this S-nitrosylation of PDI is blocked by iNOS inhibitor 1400W We investigated whether or not aberrant generation of NO through activation of iNOS mediated S-nitrosylation of PDI in reactive astrocytes following OGD/reperf...
Administration of original fl-ps-rxRNA compound results in full brain and spinal cord penetration. ps-rxRNA variant 1 (25635) in this particular assay was insufficient to achieve brain or spinal cord penetration. ps-rxRNA variants 2 and 3 (25637 and 25645, respectively) both result in uptake...