Literature DB >> 19704786

Nucleoid autonomy: An underlying mechanism of mitochondrial genetics with therapeutic potential.

Robert W Gilkerson1, Eric A Schon.   

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.

Entities:  

Keywords:  heteroplasmy; mitochondria; mitochondrial DNA; nucleoid; therapeutics

Year:  2008        PMID: 19704786      PMCID: PMC2633794          DOI: 10.4161/cib.1.1.6622

Source DB:  PubMed          Journal:  Commun Integr Biol        ISSN: 1942-0889


  19 in total

Review 1.  No sex please, we're mitochondria: a hypothesis on the somatic unit of inheritance of mammalian mtDNA.

Authors:  H T Jacobs; S K Lehtinen; J N Spelbrink
Journal:  Bioessays       Date:  2000-06       Impact factor: 4.345

Review 2.  Mitochondrial respiratory-chain diseases.

Authors:  Salvatore DiMauro; Eric A Schon
Journal:  N Engl J Med       Date:  2003-06-26       Impact factor: 91.245

3.  Human mitochondrial DNA nucleoids are linked to protein folding machinery and metabolic enzymes at the mitochondrial inner membrane.

Authors:  Yousong Wang; Daniel F Bogenhagen
Journal:  J Biol Chem       Date:  2006-07-06       Impact factor: 5.157

4.  Human prohibitin 1 maintains the organization and stability of the mitochondrial nucleoids.

Authors:  Katsumi Kasashima; Megumi Sumitani; Masaaki Satoh; Hitoshi Endo
Journal:  Exp Cell Res       Date:  2008-01-16       Impact factor: 3.905

5.  Human mitochondrial DNA is packaged with TFAM.

Authors:  Tanfis Istiaq Alam; Tomotake Kanki; Tsuyoshi Muta; Koutarou Ukaji; Yoshito Abe; Hiroshi Nakayama; Koji Takio; Naotaka Hamasaki; Dongchon Kang
Journal:  Nucleic Acids Res       Date:  2003-03-15       Impact factor: 16.971

6.  A replicating module as the unit of mitochondrial structure and functioning.

Authors:  Roderick A Capaldi; Robert Aggeler; Robert Gilkerson; George Hanson; Michelle Knowles; Andrew Marcus; Daciana Margineantu; Michael Marusich; James Murray; Devin Oglesbee; S James Remington; Rodrigue Rossignol
Journal:  Biochim Biophys Acta       Date:  2002-09-10

7.  Mitochondrial transcription factor A regulates mtDNA copy number in mammals.

Authors:  Mats I Ekstrand; Maria Falkenberg; Anja Rantanen; Chan Bae Park; Martina Gaspari; Kjell Hultenby; Pierre Rustin; Claes M Gustafsson; Nils-Göran Larsson
Journal:  Hum Mol Genet       Date:  2004-03-11       Impact factor: 6.150

8.  Selection against pathogenic mtDNA mutations in a stem cell population leads to the loss of the 3243A-->G mutation in blood.

Authors:  Harsha Karur Rajasimha; Patrick F Chinnery; David C Samuels
Journal:  Am J Hum Genet       Date:  2008-02       Impact factor: 11.025

9.  High levels of mitochondrial DNA deletions in substantia nigra neurons in aging and Parkinson disease.

Authors:  Andreas Bender; Kim J Krishnan; Christopher M Morris; Geoffrey A Taylor; Amy K Reeve; Robert H Perry; Evelyn Jaros; Joshua S Hersheson; Joanne Betts; Thomas Klopstock; Robert W Taylor; Douglass M Turnbull
Journal:  Nat Genet       Date:  2006-04-09       Impact factor: 38.330

10.  Mitochondrial nucleoids maintain genetic autonomy but allow for functional complementation.

Authors:  Robert W Gilkerson; Eric A Schon; Evelyn Hernandez; Mercy M Davidson
Journal:  J Cell Biol       Date:  2008-06-23       Impact factor: 10.539

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  2 in total

Review 1.  Mechanism of homologous recombination and implications for aging-related deletions in mitochondrial DNA.

Authors:  Xin Jie Chen
Journal:  Microbiol Mol Biol Rev       Date:  2013-09       Impact factor: 11.056

Review 2.  Mitochondrial DNA genetics and the heteroplasmy conundrum in evolution and disease.

Authors:  Douglas C Wallace; Dimitra Chalkia
Journal:  Cold Spring Harb Perspect Biol       Date:  2013-11-01       Impact factor: 10.005

  2 in total

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