ZFIN ID: ZDB-FIG-170111-9
Begay et al., 2016 - FLNC Gene Splice Mutations Cause Dilated Cardiomyopathy.. JACC. Basic to translational science   1:344-359 Full text @ JACC Basic Transl Sci
ADDITIONAL FIGURES
PHENOTYPE:
Fish:
Knockdown Reagents:
Observed In:
Stage: Protruding-mouth

Fig. 6

ltrastructural Analysis Indicates Deficiencies in Sarcomere Organization and Z-Disc Formation

Transmission electron micrographs of zebrafish hearts at 72 hpf indicate that (A) wild-type embryos had assembled several consecutive sarcomeres with clearly distinct Z-discs (black arrow), whereas flncb+p53 MO hearts (B to F) show evidence of disorganized ultrastructure. (B) Most Z-discs were diffusely stained, irregular in shape (black arrowheads), or seemingly absent (white arrowheads). Insets: Cross section through myofilament bundles indicated a normal primary organization of thick and thin filaments in hexagonal lattices in flncb-depleted hearts. (C) Sarcomere arrangement in myofibrils (m) was often nonconsecutive, with (D) multiple sarcomeres sometimes adjoined to a mass of Z-disc-like material (z). (E) Small vacuoles (v) were often present between or near the plasma membranes of adjacent cardiomyocytes. These vacuoles could be large, creating a gap between adjacent cells. (F) Autophagic vesicles (a) were observed. Scale bar: 1 ╬╝mol/l. Abbreviations as in Figure 4.

Gene Expression Details No data available
Antibody Labeling Details No data available
Phenotype Details
Fish Conditions Stage Phenotype
WT + MO2-flncb + MO4-tp53 standard conditions Protruding-mouth cardiac muscle cell autophagosome present, abnormal
Protruding-mouth cardiac muscle cell development disrupted, abnormal
Protruding-mouth cardiac muscle cell vacuolated, abnormal
Protruding-mouth cardiac myofibril assembly process quality, normal
Protruding-mouth heart cardiac myofibril disorganized, abnormal
Protruding-mouth heart Z disc disorganized, abnormal
Protruding-mouth sarcomere organization disrupted, abnormal
Acknowledgments:
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