title: Influence of a mitochondrial genetic defect on capacitative calcium entry and mitochondrial organization in the osteosarcoma cells.
abstract: Effects of T8993G mutation in mitochondrial DNA (mtDNA), associated with neurogenical muscle weakness, ataxia and retinitis pigmentosa (NARP), on the cytoskeleton, mitochondrial network and calcium homeostasis in human osteosarcoma cells were investigated. In 98% NARP and rho(0) (lacking mtDNA) cells, the organization of the mitochondrial network and actin cytoskeleton was disturbed. Capacitative calcium entry (CCE) was practically independent of mitochondrial energy status in osteosarcoma cell lines. The significantly slower Ca(2+) influx rates observed in 98% NARP and rho(0), in comparison to parental cells, indicates that proper actin cytoskeletal organization is important for CCE in these cells.
authors.affiliation: Department of Cellular Biochemistry, Nencki Institute of Experimental Biology, Pasteura 3, 02 093 Warsaw, Poland. j.szczepanowska@nencki.gov.pl
authors.forename: Joanna
authors.initials: J
authors.lastname: Szczepanowska
authors.forename: Krzysztof
authors.initials: K
authors.lastname: Zabłocki
authors.forename: Jerzy
authors.initials: J
authors.lastname: Duszyński
mesh_terms: D000199:Actins / Q000235:genetics / Q000378:metabolism; D001259:Ataxia / Q000235:genetics; D001562:Benzimidazoles; D002118:Calcium / Q000378:metabolism*; D002232:Carbocyanines; D002258:Carbonyl Cyanide m-Chlorophenyl Hydrazone / Q000494:pharmacology; D045744:Cell Line, Tumor; D003599:Cytoskeleton / Q000235:genetics / Q000378:metabolism*; D004272:DNA, Mitochondrial / Q000235:genetics* / Q000378:metabolism; D005456:Fluorescent Dyes; D016257:Fura-2; D006801:Humans; D007091:Image Processing, Computer-Assisted; D007150:Immunohistochemistry; D007476:Ionophores / Q000494:pharmacology; D008564:Membrane Potentials / Q000187:drug effects; D008856:Microscopy, Fluorescence; D017240:Mitochondrial Myopathies / Q000235:genetics*; D018908:Muscle Weakness / Q000235:genetics; D009154:Mutation; D012516:Osteosarcoma / Q000378:metabolism / Q000473:pathology; D010590:Phalloidine; D012174:Retinitis Pigmentosa / Q000235:genetics; D012235:Rhodamines; D019284:Thapsigargin / Q000494:pharmacology
chemical_list: D000199:Actins; D001562:Benzimidazoles; D002232:Carbocyanines; D004272:DNA, Mitochondrial; D005456:Fluorescent Dyes; D007476:Ionophores; D012235:Rhodamines; D010590:Phalloidine; C068624:5,5',6,6'-tetrachloro-1,1',3,3'-tetraethylbenzimidazolocarbocyanine; D002258:Carbonyl Cyanide m-Chlorophenyl Hydrazone; D019284:Thapsigargin; D002118:Calcium; D016257:Fura-2