FIGURE

Fig 3

ID

ZDB-FIG-220227-22

Publication

Berger et al., 2022 - Genetic dissection of novel myopathy models reveals a role of CapZα and Leiomodin 3 during myofibril elongation

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Fig 3

Aggregates within 4-dpf-old <italic toggle='yes'>lmod3</italic><sup><italic toggle='yes'>sa13018</italic></sup> are reminiscent of nemaline bodies formed within nemaline myopathy patients.

(A) Immunohistochemistry using antibodies against α-Actinin (green) and phalloidin staining (red) marked aggregates located close to vertical myosepta of 4-dpf-old lmod3^sa13018 homozygotes. Detected aggregates (arrowhead) were of various sizes and shapes, including rod-shaped structures (arrow). (B) Labelling of the myofibril with Lifeact-GFP (green) and the sarcolemma with mCherry-CaaX (red) confirmed aggregate (arrowhead) formation in lmod3^sa13018 homozygotes at 4 dpf, again exclusively at vertical myosepta. (C) At 4 dpf, transmission electron micrographs (TEM) of skeletal muscle from siblings showed the typical myofibril striation and well-aligned sarcomeres. Comparable sarcomeres were also present within lmod3^sa13018 homozygotes. However, close to the vertical myosepta, filament deposits reminiscent of fingerprint bodies (star), misaligned sarcomeres (arrows), and electron-dense aggregates of various sizes (arrowheads) were detected. (D) At 5 pdf, Gomori trichrome-stained sagittal sections depicted blue/purple structures close to the vertical myosepta within myofibres of lmod3^sa13018 homozygotes. Scale bar sizes are indicated.

Expression Data

Genes:	EGFP mCherry
Antibody:	Ab1-actn
Fish:	TU lmod3^{sa13018/sa13018} (TU) pc21Tg; pc22Tg lmod3^{sa13018/sa13018}; pc21Tg; pc22Tg
Anatomical Terms:	myotome sarcolemma myotome sarcomere myotome striated muscle thin filament myotome Z disc
Stage:	Day 4

Expression Detail

Antibody Labeling

Phenotype Data

Fish:	lmod3^{sa13018/sa13018} (TU) lmod3^{sa13018/sa13018}; pc21Tg; pc22Tg
Observed In:	myotome peripheral region myotome protein-containing complex myotome sarcomere myotome sarcomere organization
Stage Range:	Day 4 to Day 5

Phenotype Detail

Acknowledgments

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