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

Autor:	Josef Zamecnik, Tomas Honzik, Jana Sladkova, Olga Kostkova, Markéta Tesařová, Jana Spacilova, J. Martínek, Hana Hansikova, Jiri Zeman, M. Čapek
Rok vydání:	2015
Předmět:	Male Morphology (linguistics) Mitochondrial Diseases Mitochondrial disease Megamitochondria Infant Mitochondrion Biology medicine.disease Pathology and Forensic Medicine Cell biology Mitochondria Myoblasts Microscopy Electron Transmission Microscopy Fluorescence Structural Biology Fluorescence microscope Ultrastructure medicine Myocyte Humans Female Fragmentation (cell biology) Child
Zdroj:	Ultrastructural pathology. 39(5)
ISSN:	1521-0758
Popis:	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.
Databáze:	OpenAIRE
Externí odkaz:	https://explore.openaire.eu/search/publication?articleId=doi_dedup___::f9d5102141916ab06cb68e595b9ad9ac https://pubmed.ncbi.nlm.nih.gov/26214044 Zobrazit plný text záznamu Plný text ve formátu PDF Plný text ve formátu HTML
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