ZFIN ID: ZDB-FIG-151008-11
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. 7

Disorganized Cohnheim’s fields, swollen reticulum and altered mitochondria in col6a1ama605003 mutant fish muscle.

TEM pictures of transversal sections of muscle from wild type (WT, A1-3) and col6a1ama605003 heterozygous (HT, B1-3) and homozygous (HM, C1-3) mutants at 2 dpf, 3 wpf and 4 mpf. At 2dpf (A1, B1, C1), 3 wpf (A2, B2, C2) and 4 mpf (A3, B3, C3) in HT and HM mutants, we observed in a few muscle fibers, abnormal mitochondria with areas of the matrix devoid of electron dense material (B1-3, C1-3, arrows). In these myofibers, the abnormal mitochondria were often located in close contact with enlarged sarcoplasmic reticulum (B1-2, C1-3, arrowheads). The altered mitochondria often marked the limit between crystal-like organized (o) Cohnheim’s field and pathologic disorganized (d) myofibrils (B1-3, C1-2). In HM (C1), some fibers appeared more affected than in HT, with drastically disorganized (d) areas separated from organized myofibrils (o) with mitochondria having swollen cristae (C1-3, arrows). The sarcoplasm area of the more affected fibers appeared devoid of material (B3, C1-2, asterisks). (B3) In a few muscle fibers of HM, the reticulum appeared drastically dilated (asterisk) with numerous blebs or autophagic vacuoles localized nearby or within myofibrillar disarray foci (C2) that were also in close contact with mitochondria with enlarged cristae (C2-3, arrows). None abnormal mitochondrion, myofibril disorganization or enlarged reticulum was seen in WT (A1-3).

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 matrix morphology, abnormal
Long-pec muscle cell mitochondrion morphology, abnormal
Long-pec muscle cell sarcoplasmic reticulum increased size, abnormal
Long-pec muscle cell skeletal muscle myofibril disorganized, abnormal
Days 21-29 muscle cell mitochondrial matrix morphology, abnormal
Days 21-29 muscle cell mitochondrion morphology, abnormal
Days 21-29 muscle cell sarcoplasmic reticulum increased size, abnormal
Days 21-29 muscle cell skeletal muscle myofibril disorganized, abnormal
Adult muscle cell mitochondrial matrix morphology, abnormal
Adult muscle cell mitochondrion morphology, abnormal
Adult muscle cell sarcoplasmic reticulum increased size, abnormal
Adult muscle cell skeletal muscle myofibril disorganized, abnormal
col6a1ama605003/ama605003(TU) standard conditions Long-pec muscle cell mitochondrial crista swollen, abnormal
Long-pec muscle cell mitochondrial matrix morphology, abnormal
Long-pec muscle cell mitochondrion morphology, abnormal
Long-pec muscle cell sarcoplasm composition, abnormal
Long-pec muscle cell sarcoplasmic reticulum increased size, abnormal
Long-pec muscle cell skeletal muscle myofibril disorganized, abnormal
Days 21-29 muscle cell mitochondrial crista swollen, abnormal
Days 21-29 muscle cell mitochondrial matrix morphology, abnormal
Days 21-29 muscle cell mitochondrion morphology, abnormal
Days 21-29 muscle cell sarcoplasm composition, abnormal
Days 21-29 muscle cell sarcoplasmic reticulum increased size, abnormal
Days 21-29 muscle cell skeletal muscle myofibril disorganized, abnormal
Adult muscle cell mitochondrial crista swollen, abnormal
Adult muscle cell mitochondrial matrix morphology, abnormal
Adult muscle cell mitochondrion morphology, abnormal
Adult muscle cell sarcoplasmic reticulum increased size, 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