Analysis of Mitochondrial Network Morphology in Cultured Myoblasts from Patients with Mitochondrial Disorders. (3rd September 2015)

• Record Type: Journal Article
• Authors: Sládková, J.; Spáčilová, J.; Čapek, M.; Tesařová, M.; Hansíková, H.; Honzík, T.; Martínek, J.; Zámečník, J.; Kostková, O.; Zeman, J.
• Is Part Of: Ultrastructural pathology. Volume 39:Number 5(2015:Oct.)
• Page Start: 340
• Page End: 350
• Publication Date: 2015-09-03
• Subjects: Cristae -- Fiji -- image analysis -- mitochondrial disorders -- myoblasts -- ultrastructure; Pathology, Cellular -- Periodicals; Ultrastructure (Biology) -- Periodicals; Diagnosis, Electron microscopic -- Periodicals; Microscopy, Electron; Pathology; 616.07582
• DOI: 10.3109/01913123.2015.1054013
• Languages: English
• ISSNs: 0191-3123

Abstract

Abstract: 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.