FIGURE

Fig. 6

ID

ZDB-FIG-241204-6

Publication

Burrows et al., 2024 - RAN Translation of C9orf72-Related Dipeptide Repeat Proteins in Zebrafish Recapitulates Hallmarks of Amyotrophic Lateral Sclerosis and Identifies Hypothermia as a Therapeutic Strategy

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Fig. 6

Hypothermic-TTM reduces DPR production in 5 days postfertilization embryonic C9orf72-ALS/FTD zebrafish. Western blot and MSD poly(GP) ELISA analysis of 5 days postfertilization embryonic lysates following 72 hours of hypothermic cooling. (A, B) anti-V5-DPR immunostaining and (C, D) MSD poly(GP) ELISA analysis of sense (S5) embryonic lysates. (E, F) The anti-poly(GP) immunostaining and (G, H) MSD poly(GP) ELISA analysis of antisense (AS1) 5 days postfertilization embryonic lysates. MSD ELISA assay responses were interpolated using a GP (7) standard prior to normalization. Western blot – N = 5 to 6 clutches per treatment group; poly(GP) MSD ELISA – N = 3 clutches per treatment group. Data presented as mean ± SD. Unpaired t test, *p < 0.05, **p < 0.01, ***p < 0.001, ****p < 0.0001. ALS = amyotrophic lateral sclerosis; ELISA = enzyme-linked immunosorbent assay; FTD = frontotemporal dementia; DPR = dipeptide repeat; MSD = Meso Scale Discovery; SD = standard deviation; TTM = therapeutic temperature management. [Color figure can be viewed at www.annalsofneurology.org]

Expression Data

Expression Detail

Antibody Labeling

Phenotype Data

Phenotype Detail

Acknowledgments

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