Analysis of Mitochondrial Network Morphology in Cultured Myoblasts from Patients with Mitochondrial Disorders

Mitochondrial morphology was studied in cultivated myoblasts obtained from patients with mitochondrial disorders, including CPEO, MELAS and TMEM70 deficiency. Mitochondrial networks and ultrastructure were visualized by fluorescence microscopy and transmission electron microscopy, respectively. A he...

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Bibliographic Details
Published in:Ultrastructural pathology 2015-09, Vol.39 (5), p.340-350
Main Authors: Sládková, J., Spáčilová, J., Čapek, M., Tesařová, M., Hansíková, H., Honzík, T., Martínek, J., Zámečník, J., Kostková, O., Zeman, J.
Format: Article
Language:English
Subjects:
Child
Cristae
Female
Fiji
Humans
image analysis
Infant
Male
Microscopy, Electron, Transmission
Microscopy, Fluorescence
Mitochondria - ultrastructure
Mitochondrial Diseases - pathology
mitochondrial disorders
myoblasts
Myoblasts - ultrastructure
ultrastructure
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Summary:Mitochondrial morphology was studied in cultivated myoblasts obtained from patients with mitochondrial disorders, including CPEO, MELAS and TMEM70 deficiency. Mitochondrial networks and ultrastructure were visualized by fluorescence microscopy and transmission electron microscopy, respectively. A heterogeneous picture of abnormally sized and shaped mitochondria with fragmentation, shortening, and aberrant cristae, lower density of mitochondria and an increased number of "megamitochondria" were found in patient myoblasts. Morphometric Fiji analyses revealed different mitochondrial network properties in myoblasts from patients and controls. The small number of cultivated myoblasts required for semiautomatic morphometric image analysis makes this tool useful for estimating mitochondrial disturbances in patients with mitochondrial disorders.
ISSN:0191-3123
1521-0758
DOI:10.3109/01913123.2015.1054013