FIGURE

Fig. 7

ID

ZDB-FIG-180720-14

Publication

Burns et al., 2018 - Variants in EXOSC9 Disrupt the RNA Exosome and Result in Cerebellar Atrophy with Spinal Motor Neuronopathy

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Fig. 7

Knockdown of exosc9 in Zebrafish Causes Abnormal Neuromuscular Development

(A) Whole-mount immunofluorescence of the pan-neuronal marker HuC shows that the midbrain (^∗) appears abnormal and the cerebellum (#) and hindbrain (+) are absent in representative exosc9 morphants and crispants compared with uninjected controls.

(B) Islet1:GFP transgenic morphant and crispant zebrafish have absent cranial posterior nerve V (Vp).

(C) Whole-mount immunofluorescence of synaptic vesicle 2 (SV2, motorneurons, green) and α-bungarotoxin (neuromuscular junctions, red) shows that motoneurons and neuromuscular junctions failed to properly develop in exosc9 morphants and crispants compared with uninjected controls (white arrows).

(D) Phalloidin staining shows that muscle failed to develop properly. Fibers were sparser, more spread out, and irregular in the exosc9 morphants and crispants. All experiments were performed in 48 hpf zebrafish.

Expression Data

Expression Detail

Antibody Labeling

Phenotype Data

Fish:	rw0Tg + CRISPR2-exosc9 rw0Tg + MO1-exosc9
Knockdown Reagents:	CRISPR2-exosc9 MO1-exosc9
Observed In:	cerebellum cranial nerve V hindbrain midbrain motor neuron trunk musculature neuromuscular junction trunk musculature skeletal muscle cell
Stage:	Long-pec

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 @ Am. J. Hum. Genet.