ZFIN ID: ZDB-FIG-151008-13
Radev et al., 2015 - A TALEN-Exon Skipping Design for a Bethlem Myopathy Model in Zebrafish. PLoS One   10:e0133986 Full text @ PLoS One
ADDITIONAL FIGURES
PHENOTYPE:
Fish:
Observed In:
Stage Range: Long-pec to Adult

Fig. 9

Altered ultrastructure of mitochondria in col6a1ama605003 mutant fish myofibers.

TEM pictures of sagittal sections of muscles from wild type (WT, A1-4), heterozygous (HT, B1-4) and homozygous (HM, C1-4) col6a1ama605003 mutants, at 2 dpf (A1, B1, C1), 3 wpf (A2, B2, C2), and 4 mpf (A3-4, B3-4, C3-4). In WT, the mitochondria constantly exhibited a dense and well-delineated lamination of the inner membrane and a well-defined outer membrane (A), attesting of the good quality of the fixation-inclusion procedures. In HT and HM muscles, the morphology of mitochondria was diversiform. Similarly to observations in Figs 7 and 8, some mitochondria presented a matrix partly cleared, or even devoid, of electron dense material (B, C arrow) and with severely dilated external membrane detached from the inner condensed membrane (B2-3, C3-4, arrows). These abnormalities were often associated with figures of vacuole/autophagic vesicles (C2, C4, asterisk). In the same area of HT or HM sections, we often observed a normal mitochondrion close to another one exhibiting either swollen cristae (B4, C4, arrows), or even cristae reduced to a few vesicles (B1-2, C2). In a few mitochondria the cristae were even ultra-condensed to a dense core separated from the outer membrane by a vesicle (arrows, B3.1–2, C1). The abnormal mitochondria most often bounded the limits between the crystal-like organized (o) and disorganized (d) myofibrils.

Gene Expression Details No data available
Antibody Labeling Details No data available
Phenotype Details
Fish Conditions Stage Phenotype
col6a1ama605003/+(TU) standard conditions Long-pec muscle cell mitochondrial crista morphology, abnormal
Long-pec muscle cell mitochondrial crista swollen, abnormal
Long-pec muscle cell mitochondrial matrix morphology, abnormal
Long-pec muscle cell mitochondrial outer membrane detached from muscle cell mitochondrial inner membrane, abnormal
Long-pec muscle cell mitochondrion morphology, abnormal
Long-pec muscle cell skeletal muscle myofibril disorganized, abnormal
Days 21-29 muscle cell mitochondrial crista morphology, abnormal
Days 21-29 muscle cell mitochondrial crista swollen, abnormal
Days 21-29 muscle cell mitochondrial matrix morphology, abnormal
Days 21-29 muscle cell mitochondrial outer membrane detached from muscle cell mitochondrial inner membrane, abnormal
Days 21-29 muscle cell mitochondrion morphology, abnormal
Days 21-29 muscle cell skeletal muscle myofibril disorganized, abnormal
Adult muscle cell mitochondrial crista morphology, abnormal
Adult muscle cell mitochondrial crista swollen, abnormal
Adult muscle cell mitochondrial matrix morphology, abnormal
Adult muscle cell mitochondrial outer membrane detached from muscle cell mitochondrial inner membrane, abnormal
Adult muscle cell mitochondrion morphology, abnormal
Adult muscle cell skeletal muscle myofibril disorganized, abnormal
col6a1ama605003/ama605003(TU) standard conditions Long-pec muscle cell mitochondrial crista morphology, abnormal
Long-pec muscle cell mitochondrial crista swollen, abnormal
Long-pec muscle cell mitochondrial matrix morphology, abnormal
Long-pec muscle cell mitochondrial outer membrane detached from muscle cell mitochondrial inner membrane, abnormal
Long-pec muscle cell mitochondrion morphology, abnormal
Long-pec muscle cell skeletal muscle myofibril disorganized, abnormal
Days 21-29 muscle cell mitochondrial crista morphology, abnormal
Days 21-29 muscle cell mitochondrial crista swollen, abnormal
Days 21-29 muscle cell mitochondrial matrix morphology, abnormal
Days 21-29 muscle cell mitochondrial outer membrane detached from muscle cell mitochondrial inner membrane, abnormal
Days 21-29 muscle cell mitochondrion morphology, abnormal
Days 21-29 muscle cell skeletal muscle myofibril disorganized, abnormal
Adult muscle cell mitochondrial crista morphology, abnormal
Adult muscle cell mitochondrial crista swollen, abnormal
Adult muscle cell mitochondrial matrix morphology, abnormal
Adult muscle cell mitochondrial outer membrane detached from muscle cell mitochondrial inner membrane, abnormal
Adult muscle cell mitochondrion morphology, abnormal
Adult muscle cell skeletal muscle myofibril disorganized, abnormal
Acknowledgments:
ZFIN wishes to thank the journal PLoS One for permission to reproduce figures from this article. Please note that this material may be protected by copyright. Full text @ PLoS One