title: Analysis of Mitochondrial Network Morphology in Cultured Myoblasts from Patients with Mitochondrial Disorders.
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.
authors.affiliation: a Department of Pediatrics and Adolescent Medicine, First Faculty of Medicine , Charles University in Prague and General University Hospital in Prague , Prague , Czech Republic .
authors.forename: J
authors.initials: J
authors.lastname: Sládková
authors.affiliation: a Department of Pediatrics and Adolescent Medicine, First Faculty of Medicine , Charles University in Prague and General University Hospital in Prague , Prague , Czech Republic .
authors.forename: J
authors.initials: J
authors.lastname: Spáčilová
authors.affiliation: b Department of Biomathematics , Institute of Physiology, The Czech Academy of Sciences , Prague , Czech Republic .
authors.forename: M
authors.initials: M
authors.lastname: Čapek
authors.affiliation: a Department of Pediatrics and Adolescent Medicine, First Faculty of Medicine , Charles University in Prague and General University Hospital in Prague , Prague , Czech Republic .
authors.forename: M
authors.initials: M
authors.lastname: Tesařová
authors.affiliation: a Department of Pediatrics and Adolescent Medicine, First Faculty of Medicine , Charles University in Prague and General University Hospital in Prague , Prague , Czech Republic .
authors.forename: H
authors.initials: H
authors.lastname: Hansíková
authors.affiliation: a Department of Pediatrics and Adolescent Medicine, First Faculty of Medicine , Charles University in Prague and General University Hospital in Prague , Prague , Czech Republic .
authors.forename: T
authors.initials: T
authors.lastname: Honzík
authors.affiliation: c Institute of Histology and Embryology, First Faculty of Medicine, Charles University in Prague and General University Hospital in Prague , Prague , Czech Republic , and.
authors.forename: J
authors.initials: J
authors.lastname: Martínek
authors.affiliation: d Department of Pathology and Molecular Medicine , University Hospital in Motol , Prague , Czech Republic.
authors.forename: J
authors.initials: J
authors.lastname: Zámečník
authors.affiliation: a Department of Pediatrics and Adolescent Medicine, First Faculty of Medicine , Charles University in Prague and General University Hospital in Prague , Prague , Czech Republic .
authors.forename: O
authors.initials: O
authors.lastname: Kostková
authors.affiliation: a Department of Pediatrics and Adolescent Medicine, First Faculty of Medicine , Charles University in Prague and General University Hospital in Prague , Prague , Czech Republic .
authors.forename: J
authors.initials: J
authors.lastname: Zeman
mesh_terms: D002648:Child; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D046529:Microscopy, Electron, Transmission; D008856:Microscopy, Fluorescence; D008928:Mitochondria / Q000648:ultrastructure*; D028361:Mitochondrial Diseases / Q000473:pathology*; D032446:Myoblasts / Q000648:ultrastructure*
keywords: Cristae; Fiji; image analysis; mitochondrial disorders; myoblasts; ultrastructure