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

0449498 - FGÚ 2016 RIV US eng J - Journal Article
Sládková, J. - Spáčilová, J. - Čapek, Martin - Tesařová, M. - Hansíková, H. - Honzík, T. - Martínek, J. - Zámečník, J. - Kostková, O. - Zeman, J.
Analysis of Mitochondrial Network Morphology in Cultured Myoblasts from Patients with Mitochondrial Disorders.
Ultrastructural Pathology. Roč. 39, č. 5 (2015), s. 340-350. ISSN 0191-3123. E-ISSN 1521-0758
R&D Projects: GA ČR(CZ) GB14-36804G; GA MŠMT(CZ) LH13028
Institutional support: RVO:67985823
Keywords : cristae * Fiji * image analysis * mitochondrial disorders * myoblasts * ultrastructure
Subject RIV: EA - Cell Biology
Impact factor: 0.828, year: 2015

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
Permanent Link: http://hdl.handle.net/11104/0251051