(A) Embryos obtained from an in-cross of heterozygous mia40a+/- siblings in the Tg(Xla.Eef1a1:mlsEGFP) background were stained with phalloidin to visualize actin filaments of the skeletal muscle at 4 dpf. Abnormal, enlarged mitochondrial structures are found in the skeletal muscles of the mia40a mutants (arrowheads). To better visualize the GFP-positive inclusions, a magnified image is shown on the side. Images are maximum projections. Scale bar, 50 μm. All images are lateral views, anterior to the left. (B) wild-type and mia40a-/- larvae in the Tg(Xla.Eef1a1:mlsEGFP) background were stained with an anti-Tomm20 antibody to mark the outer mitochondrial membrane (OMM). DNA was counterstained with DAPI. Single plane sections are shown. The arr...
<p>Mit indicates mitochondria. T indicates triad. Representative electron microscopy images (D,E,F,)...
<p>A–D- Images (A, C) and quantification (B, D) of mitochondrial immunolabeling with Complex V (anti...
<p>(A) Mass of gastrocnemius muscles reported to body weight of the mice (n = 10–15 per group). (B) ...
The mitochondrial DNA A3243G mutation causes neuromuscular disease. To investigate the muscle-specif...
BACKGROUND AND PURPOSE: The pathogenesis of mitochondrial disease (MD) involves the disruption of ce...
We have generated an animal model for mitochondrial myopathy by disrupting the gene for mitochondria...
A girl, who died at 14 years of age from a rapidly progressive mitochondrial myopathy, was found to ...
Thoracic muscles from Oregon-R wild-type flies plus mutant flies ATPsynC1rev18.3 and ATPsynC1rev24.2...
Background: Mitochondrial diseases display a heterogeneous spectrum of clinical phenotypes and there...
Central core disease (CCD), congenital fibre type disproportion (CFTD), and nemaline myopathy (NM) a...
<p>(A–C) Quadricep muscles from 9–10 month-old wild-type and VCP<sup>R155H/+</sup> knock-in mice wer...
The mitochondrial import machinery consists of several translocases. By gene trap mutagenesis, a kno...
Item does not contain fulltextBACKGROUND: Mitochondrial diseases display a heterogeneous spectrum of...
<p>DAPI staining of representative EDL and soleus myofibres from wild-type (WT) <i>lmna<sup>+/+</sup...
AbstractMyofibrillar myopathies (MFM) are characterised by focal myofibrillar destruction and accumu...
<p>Mit indicates mitochondria. T indicates triad. Representative electron microscopy images (D,E,F,)...
<p>A–D- Images (A, C) and quantification (B, D) of mitochondrial immunolabeling with Complex V (anti...
<p>(A) Mass of gastrocnemius muscles reported to body weight of the mice (n = 10–15 per group). (B) ...
The mitochondrial DNA A3243G mutation causes neuromuscular disease. To investigate the muscle-specif...
BACKGROUND AND PURPOSE: The pathogenesis of mitochondrial disease (MD) involves the disruption of ce...
We have generated an animal model for mitochondrial myopathy by disrupting the gene for mitochondria...
A girl, who died at 14 years of age from a rapidly progressive mitochondrial myopathy, was found to ...
Thoracic muscles from Oregon-R wild-type flies plus mutant flies ATPsynC1rev18.3 and ATPsynC1rev24.2...
Background: Mitochondrial diseases display a heterogeneous spectrum of clinical phenotypes and there...
Central core disease (CCD), congenital fibre type disproportion (CFTD), and nemaline myopathy (NM) a...
<p>(A–C) Quadricep muscles from 9–10 month-old wild-type and VCP<sup>R155H/+</sup> knock-in mice wer...
The mitochondrial import machinery consists of several translocases. By gene trap mutagenesis, a kno...
Item does not contain fulltextBACKGROUND: Mitochondrial diseases display a heterogeneous spectrum of...
<p>DAPI staining of representative EDL and soleus myofibres from wild-type (WT) <i>lmna<sup>+/+</sup...
AbstractMyofibrillar myopathies (MFM) are characterised by focal myofibrillar destruction and accumu...
<p>Mit indicates mitochondria. T indicates triad. Representative electron microscopy images (D,E,F,)...
<p>A–D- Images (A, C) and quantification (B, D) of mitochondrial immunolabeling with Complex V (anti...
<p>(A) Mass of gastrocnemius muscles reported to body weight of the mice (n = 10–15 per group). (B) ...