title: Cytoplasmic hybrid (cybrid) cell lines as a practical model for mitochondriopathies.
abstract: Cytoplasmic hybrid (cybrid) cell lines can incorporate human subject mitochondria and perpetuate its mitochondrial DNA (mtDNA)-encoded components. Since the nuclear background of different cybrid lines can be kept constant, this technique allows investigators to study the influence of mtDNA on cell function. Prior use of cybrids has elucidated the contribution of mtDNA to a variety of biochemical parameters, including electron transport chain activities, bioenergetic fluxes, and free radical production. While the interpretation of data generated from cybrid cell lines has technical limitations, cybrids have contributed valuable insight into the relationship between mtDNA and phenotype alterations. This review discusses the creation of the cybrid technique and subsequent data obtained from cybrid applications.
authors.affiliation: Department of Neurology and the University of Kansas Alzheimer's Disease Center, University of Kansas Medical Center, Kansas City, KS, United States of America.
authors.forename: Heather M
authors.initials: HM
authors.lastname: Wilkins
authors.affiliation: Department of Neurology and the University of Kansas Alzheimer's Disease Center, University of Kansas Medical Center, Kansas City, KS, United States of America.
authors.forename: Steven M
authors.initials: SM
authors.lastname: Carl
authors.affiliation: Department of Neurology and the University of Kansas Alzheimer's Disease Center, University of Kansas Medical Center, Kansas City, KS, United States of America; Departments of Molecular and Integrative Physiology and Biochemistry and Molecular Biology, University of Kansas Medical Center, Kansas City, KS, United States of America. Electronic address: rswerdlow@kumc.edu.
authors.forename: Russell H
authors.initials: RH
authors.lastname: Swerdlow
mesh_terms: D002459:Cell Fusion; D002460:Cell Line; D004272:DNA, Mitochondrial / Q000235:genetics / Q000378:metabolism; D006801:Humans; D008928:Mitochondria / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D028361:Mitochondrial Diseases / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D008954:Models, Biological
chemical_list: D004272:DNA, Mitochondrial
keywords: Cybrid; Mitochondria; Mitochondrial DNA; Rho zero