Research article
Superoxide dismutase 1 encoding mutations linked to ALS adopts a spectrum of misfolded states
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* Corresponding author: David R Borchelt borchelt@mbi.ufl.edu
1 Department of Neuroscience, McKnight Brain Institute, University of Florida, Gainesville, FL 32610, USA
2 Department of Neuroscience, Mayo Clinic College of Medicine, 4500 San Pablo Rd, Jacksonville, FL 32224, USA
Molecular Neurodegeneration 2011, 6:77 doi:10.1186/1750-1326-6-77
Published: 17 November 2011Additional files
Additional file 1:
Supplementary Figures S1-8; Supplementary Table 1. Suppl. Figure 1. The majority of SOD1 in transiently-transfected HEK293FT transfected cells is soluble in non-ionic detergent. Suppl. Figure 2. Formic acid treatment does not allow visualization of inclusions using a hSOD1 antibody. Suppl. Figure 3. TK negative cells transfected with SOD1 constructs for 48 hours and stained for human SOD1. Suppl. Figure 4. HEK293FT cells expressing a SOD1::YFP variant accumulate detergent-insoluble aggregates. Suppl. Figure 5. Digitonin treatment in TK negative cells reveals a punctate pattern of SOD1 immunoreactivity in cells expressing either WT or A4V SOD1. Suppl. Figure 6. Digitonin extracts a portion of the detergent-soluble hSOD1 protein expressed in HEK293FT cells. Supplemental Figure 7. EtBr uptake assay of cell permeability in HEK293FT cells expressing mutant and WT YFP fusion proteins. Suppl. Figure 8. Assessment of cell death in CHO cells expressing WT and A4V SOD::YFP fusion proteins. Supplementary Table 1. Summary of previous findings with conformationally-restricted antibodies [4,5,15,17,26,27,39,41-43,51-56].
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