title: Nucleoid autonomy: An underlying mechanism of mitochondrial genetics with therapeutic potential.
abstract: Emerging research shows that the packaging of mitochondrial DNA (mtDNA) into protein-DNA assemblies called nucleoids confers higher-order organization to the mitochondrial genome. Studies of nucleoid composition, structure and dynamics reveal the mitochondrial nucleoid to be tightly regulated in its genetic autonomy, macromolecular organization and distribution throughout the cell. Our recent research shows that mitochondrial nucleoids are self-contained genetic entities that do not exchange mtDNAs with each other frequently. This suggests that the genetic composition of a cell's nucleoids will be the key determinant of the cell's mtDNA dynamics, and provides a mechanistic basis for therapeutic methods to rescue dysfunction due to mutations in mtDNA.
authors.affiliation: Department of Neurology; College of Physicians and Surgeons; Columbia University; New York, New York USA.
authors.forename: Robert W
authors.initials: RW
authors.lastname: Gilkerson
authors.forename: Eric A
authors.initials: EA
authors.lastname: Schon
references.citation: Nat Genet. 2006 May;38(5):515-7
references.pmid: 16604074
references.citation: Ann Neurol. 2004 Nov;56(5):662-9
references.pmid: 15389892
references.citation: Nat Genet. 2006 May;38(5):518-20
references.pmid: 16604072
references.citation: Proc Natl Acad Sci U S A. 1992 Dec 1;89(23):11164-8
references.pmid: 1454794
references.citation: J Cell Sci. 2004 Jun 1;117(Pt 13):2653-62
references.pmid: 15138283
references.citation: Biochim Biophys Acta. 2002 Sep 10;1555(1-3):192-5
references.pmid: 12206914
references.citation: Nucleic Acids Res. 2003 Mar 15;31(6):1640-5
references.pmid: 12626705
references.citation: Mol Biol Cell. 2007 Sep;18(9):3225-36
references.pmid: 17581862
references.citation: Proc Natl Acad Sci U S A. 1977 Apr;74(4):1348-52
references.pmid: 266177
references.citation: Hum Mol Genet. 2004 May 1;13(9):935-44
references.pmid: 15016765
references.citation: J Biol Chem. 2006 Sep 1;281(35):25791-802
references.pmid: 16825194
references.citation: Am J Hum Genet. 2008 Feb;82(2):333-43
references.pmid: 18252214
references.citation: Bioessays. 2000 Jun;22(6):564-72
references.pmid: 10842310
references.citation: Nat Genet. 1996 Oct;14(2):146-51
references.pmid: 8841183
references.citation: Mitochondrion. 2002 May;1(5):425-35
references.pmid: 16120295
references.citation: N Engl J Med. 2003 Jun 26;348(26):2656-68
references.pmid: 12826641
references.citation: Exp Cell Res. 2008 Mar 10;314(5):988-96
references.pmid: 18258228
references.citation: J Cell Biol. 2008 Jun 30;181(7):1117-28
references.pmid: 18573913
references.citation: BMC Biol. 2004 May 24;2:9
references.pmid: 15157274