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Results: 6

1.
Fig. 6.

Fig. 6. From: Localization of a toxic form of superoxide dismutase 1 protein to pathologically affected tissues in familial ALS.

Mutant SOD1 human spinal cords show distinct skein-like intracellular inclusions immunoreactive with C4F6. All SOD1A4V cases examined (Upper panels) show skein-like inclusions in motor neurons. Non-SOD1 fALS, sporadic ALS, other neurological diseases, and nondiseased controls (Lower panels) show diffuse staining and do not exhibit skein-like inclusions. (Scale bar: 50 μm.)

Terrell E. Brotherton, et al. Proc Natl Acad Sci U S A. 2012 April 3;109(14):5505-5510.
2.
Fig. 5.

Fig. 5. From: Localization of a toxic form of superoxide dismutase 1 protein to pathologically affected tissues in familial ALS.

C4F6 detects non-G93A hSOD1 mutants. CHO cells transiently expressing hSOD1G93A, hSOD1A4V, hSOD1G37R, hSOD1G93C, and hSOD1WT were stained with C4F6 (red) or a human SOD1-specific antibody (red) to demonstrate hSOD1 expression. DAPI denotes nuclei (blue). C4F6 detected hSOD1G93A, hSOD1A4V, and hSOD1G37R, but not hSOD1G93C or hSOD1WT. (Scale bar: 50 μm.) Independent experiments were performed three times.

Terrell E. Brotherton, et al. Proc Natl Acad Sci U S A. 2012 April 3;109(14):5505-5510.
3.
Fig. 2.

Fig. 2. From: Localization of a toxic form of superoxide dismutase 1 protein to pathologically affected tissues in familial ALS.

Both disease affected and unaffected hSOD1G93A tissue is C4F6-immunoreactive when denatured through SDS/PAGE. (A) C4F6 is immunoreactive with both denatured dorsal (D) and ventral (V) hSOD1G93A spinal cord. C4F6 does not react with dorsal or ventral cord from either C57/BL6 or hSOD1WT mice. (B) Probing with a pan-SOD1 antibody demonstrates hSOD1 presence; mouse SOD1 (Lower band) runs slightly faster than human SOD1 on SDS/PAGE. Independent experiments were performed a minimum of three times.

Terrell E. Brotherton, et al. Proc Natl Acad Sci U S A. 2012 April 3;109(14):5505-5510.
4.
Fig. 4.

Fig. 4. From: Localization of a toxic form of superoxide dismutase 1 protein to pathologically affected tissues in familial ALS.

C4F6 does not robustly detect hydrophobic SOD1. Spinal cord from 135-d hSOD1G93A mouse separated by hydrophobic interaction chromatography and visualized through immunoblot following denaturing SDS/PAGE. C4F6 (Upper) detects the input (lane 1) and flow through not caught on the column (2, 3) but only weakly detects hydrophobic protein eluted off the column (4–6). A pan-SOD1 antibody (Lower) demonstrates that hydrophobic SOD1 is present in the eluate. Blot is representative of duplicate experiments.

Terrell E. Brotherton, et al. Proc Natl Acad Sci U S A. 2012 April 3;109(14):5505-5510.
5.
Fig. 3.

Fig. 3. From: Localization of a toxic form of superoxide dismutase 1 protein to pathologically affected tissues in familial ALS.

C4F6-immunoreactive protein is easily soluble. Dorsal (odd-numbered lanes) and ventral (even-numbered lanes) spinal cord from an end-stage hSOD1G93A mouse extracted in buffer without detergent (lanes 1 and 2), with 1.5% Nonidet P-40 (3, 4), with 1.5% Nonidet P-40 and 2% SDS (5, 6), with 1.5% Nonidet P-40, 2% SDS, and 6 M urea (7, 8), and with the remaining pellet (9, 10). Lane 11 is 215-d hSOD1WT; lane 12 is 70-d hSOD1G93A. Protein was separated through denaturing SDS/PAGE and transferred to PVDF membrane for immunoblot. Upper was probed with C4F6, and Lower was probed with a pan-SOD1 antibody. Note the relatively equal amounts of mutant hSOD1 protein in dorsal vs. ventral spinal cord, in contradistinction to the C4F6 immunoreactivity seen in tissue sections. Independent experiments were performed in duplicate.

Terrell E. Brotherton, et al. Proc Natl Acad Sci U S A. 2012 April 3;109(14):5505-5510.
6.
Fig. 1.

Fig. 1. From: Localization of a toxic form of superoxide dismutase 1 protein to pathologically affected tissues in familial ALS.

C4F6 is selective for pathologically affected tissue types in ALS rodents. Spinal cord sections from 47-d hSOD1G93A (A and B) and 215-d hSOD1WT (C and D) mice. A and C are stained with the C4F6 antibody and B and D are stained with the pan-SOD1 antibody. (A) Note C4F6 staining of anterior horn cells and ventral root in the ventral spinal cord and the relative lack of staining in the dorsal spinal cord and dorsal root in hSOD1G93A tissue. C4F6 did not stain the spinal cord from hSOD1WT (C). Inset in C further demonstrates this lack of immunoreactivity with a high power image of the hSOD1WT ventral horn stained with C4F6. Staining with the pan-SOD1 antibody demonstrates the diffuse presence of human mutant (B) and wild-type (D) hSOD1 protein. Spinal cord from SOD1 G93A mouse (E) and rat (F) demonstrates that C4F6 stains only a subset of motor neurons within pathologically affected tissue. Furthermore, C4F6 stains rat hSOD1G93A ventral root (G) but not dorsal root (H). Inset in G shows a high power image of ventral root axons showing staining of some, but not all, axons; Inset in H demonstrates minimal staining of dorsal root axons. IJ demonstrate that C4F6 does not recognize mutant SOD1 protein in nonaffected SOD1G93A dorsal root ganglia (I), despite the presence of human mutant SOD1 identified with the pan-SOD1 antibody (J). (Scale bars: E, 50 μm; FJ, 100 μm; all other, 200 μm.) Independent experiments were performed a minimum of three times.

Terrell E. Brotherton, et al. Proc Natl Acad Sci U S A. 2012 April 3;109(14):5505-5510.

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