FIGURE

Figure 1

ID

ZDB-FIG-230711-20

Publication

Lescouzères et al., 2023 - A multilevel screening pipeline in zebrafish identifies therapeutic drugs for GAN

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Figure 1

The gan zebrafish mimics the loss of motility described in GAN patients

Schematic of the behavioral and cellular defects described in the gan zebrafish model. Underlying the loss of motility in the gan zebrafish, the architecture of the spinal cord is remodeled with shortening and/or absence of MN axons (in green, primary MNs; in red, secondary MNs) and loss of neuromuscular junctions (NMJ). The neuromuscular phenotype is accompanied by a change in the shape of myofibers, which adapt a “U‐shape” instead of a normal “V‐shape.”

The percentage of shorter pMN axons (< 70 μm) per fish is significantly higher in gan morphants (n = 20) than in noninjected WT (n = 20) at 48 hpf.

Representative images for the neuromuscular junctions (znp1: green; αBTX: α‐bungarotoxin: red) in WT and gan morphants at 48 hpf. Note the shorter pMN axons and sparse AChR clusters in gan morphants.

Representation of the cumulative tracking of the spontaneous locomotion of 5‐day‐old larvae for 1 h, in noninjected and MO‐injected animals.

Quantitative measures of the traveled distance (m: meter) show total loss of locomotion in 79.2% of gan morphant; n = 48 (WT), n = 48 (MO).

Data information: (B, E) Each dot represents individual larvae; *P < 0.05, ****P < 0.0001. In the absence of normality of distribution of the data, a nonparametric Mann–Whitney U test was applied. Data are represented as means ± SEM. (C) Scale bar represents a length of 100 μm.

Expression Data

Expression Detail

Antibody Labeling

Phenotype Data

Phenotype Detail

Acknowledgments

This image is the copyrighted work of the attributed author or publisher, and ZFIN has permission only to display this image to its users. Additional permissions should be obtained from the applicable author or publisher of the image. Full text @ EMBO Mol. Med.