Human mtDNA sublimons resemble rearranged mitochondrial genoms found in pathological states

Human Molecular Genetics

Volumn 9, Issue 19, 2000, Pages 2821-2835

  Kajander, Olli A ^a,b   Rovio, Anja T ^a   Majamaa, Kari ^c   Poulton, Joanna ^d   Spelbrink, Johannes N ^a   Holt, Ian J ^e   Karhunen, Pekka J ^b   Jacobs, Howard T ^a,f  

^a UNIVERSITY OF TAMPERE   (Finland)

^b TAMPERE UNIVERSITY OF TECHNOLOGY   (Finland)

^c UNIVERSITY OF OULU   (Finland)

^d UNIVERSITY OF OXFORD   (United Kingdom)

^e UNIVERSITY OF CAMBRIDGE   (United Kingdom)

^f UNIVERSITY OF GLASGOW   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

DNA BINDING PROTEIN; MITOCHONDRIAL DNA;

ADULT; AGED; ARTICLE; CELL LEVEL; CHILD; CONTROLLED STUDY; DNA CONFORMATION; DNA DETERMINATION; DNA STRAND BREAKAGE; ENCEPHALOMYOPATHY; EXTRAOCULAR MUSCLE; FEMALE; FETUS; GENE REARRANGEMENT; GENETIC RECOMBINATION; GENETIC VARIABILITY; HUMAN; HUMAN CELL; HUMAN TISSUE; INFANT; MALE; MITOSIS; MOLECULAR CLONING; MYOPATHY; NUCLEOTIDE REPEAT; OPHTHALMOPLEGIA; OXIDATIVE STRESS; PLANT; PRIORITY JOURNAL; PROTEIN DNA BINDING; PROTEIN PROTEIN INTERACTION; SPECIES DIFFERENCE; TISSUE LEVEL;

ADULT; AGED; AGED, 80 AND OVER; AGING; BASE SEQUENCE; CHROMOSOME BREAKAGE; CLONING, MOLECULAR; DNA, MITOCHONDRIAL; ELECTROPHORESIS, AGAR GEL; FEMALE; FLUORESCENCE; GENE DOSAGE; GENE DUPLICATION; HUMANS; MALE; MIDDLE AGED; MITOCHONDRIA; MUTAGENESIS; MYOCARDIUM; NUCLEIC ACID CONFORMATION; ORGAN SPECIFICITY; OXIDATIVE STRESS; POLYMERASE CHAIN REACTION; RECOMBINATION, GENETIC; SEQUENCE DELETION; TUMOR CELLS, CULTURED;

EID: 0034703868     PISSN: 09646906     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (49)

1. Mitochondrial genetics and human disease
2. Mitochondria in neuromuscular disorders
3. Mitochondrial disorders
4. Spectrum of mitochondrial DNA rearrangements in the Pearson marro-pancreas syndrome
5. Clonal expansion of mitochondrial DNA with multiple deletions in autosomal dominant progressive external ophthalmoplegia
6. Families of mtDNA re-arrangements can be detected in patients with mtDNA deletions: Duplications may be a transient intermediate form
7. An autosomal dominant disorder with multiple deletions of mitochondrial DNA starting at the D-loop region
8. Autosomal dominant progressive external ophthalmoplegia: Distribution of multiple mitochondrial DNA deletions
9. Partial depletion and multiple deletions of muscle mtDNA in familial MNGIE syndrome
10. Multiple mtDNA deletions features in autosomal dominant and recessive diseases suggest distinct pathogeneses
11. Analysis of multiple mitochondrial DNA deletions in inclusion body myositis
12. Multiple mitochondrial DNA deletions in hereditary inclusion body myopathy
13. Multiple mtDNA deletions: Clinical and molecular correlations
14. Long-extension PCR to detect deleted mitochondrial DNA molecules is compromised by technical artefacts
15. Mitochondrial DNA deletions in cardiomyopathies
16. Detection and analysis of mitochondrial DNA deletions by whole genome PCR
17. The dangers of extended PCR in the clinic
18. Mitochondrial genome mutation in cell death and aging
19. The mitochondrial theory of aging: Do damaged mitochondria accumulate by delayed degradation?
20. Tissue-specific distribution of multiple mitochondrial DNA rearrangements during human aging
21. Mitochondrial DNA rearrangements in aging human brain and in situ PCR of mtDNA
22. Aging and mitochondrial
23. Whole mitochondrial genome amplification reveals basal level multiple deletions in mtDNA of patients with dilated cardiomyopathy
24. Evolution of plant mitochondrial genomes via substoichiometric intermediates
25. Involvement of two different urf-s related mitochondrial sequences in the molecular evolution of the CMS-specific S-Pcf locus in petunia
26. Tissue-specific selection for different mtDNA genotypes in heteroplasmic mice
27. Detection and characterization of mitochondrial DNA rearrangements in Pearson and Kearns-Sayre syndromes by long PCR
28. Duplication and triplication with staggered breakpoints in human mitochondrial DNA
29. Nucleus-driven multiple large-scale deletions of the human mitochondrial genome: A new autosomal dominant disease
30. Nucleus-driven mutations of human mitochondrial DNA
31. Termination of transcription in human mitochondria: Identification and purification of a DNA binding protein factor that promotes termination
32. The human mitochondrial transcription termination factor (mTERF) is a multizipper protein but binds to DNA as a monomer, with evidence pointing to intramolecular leucine zipper interactions
33. Protein binding to a single termination-associated sequence in the mitochondrial DNA D-loop region
34. Rat mitochondrial mtDNA-binding proteins to inter-specifically conserved sequences in the displacement loop region of vertebrate mtDNAs
35. Multiple protein-binding sites in the TAS-region of human and rat mitochondrial DNA
36. Two distinct, sequence-specific DNA-binding proteins interact independently with the major replication pause region of sea urchin mtDNA
37. Direct regulation of mitochondrial RNA synthesis by thyroid hormone
38. In vivo mitochondrial DNA-protein interactions in sea urchin eggs and embryos
39. Preferential amplification and phenotypic selection in a population of deleted and wild-type mitochondrial DNA in cultured cells
40. Rearrangements of human mitochondrial DNA (mtDNA): New insights into the regulation of mtDNA copy number and gene expression
41. Behaviour of a population of partially duplicated mitochondrial DNA molecules in cell culture: Segregation, maintenance and recombination dependent upon nuclear background
42. Maintenance of human rearranged mitochondrial DNAs in long-term cultured transmitochondrial cell lines
43. Coupled leading- and lagging-strand synthesis of mammalian mitochondrial DNA
44. Thymidine phosphorylase gene mutations in MNGIE, a human mitochondrial disorder
45. Role of adenine nucleotide translocator 1 in mtDNA maintenance
46. A proposed refinement of the mitochondrial free radical theory of aging
47. Human cells lacking mtDNA: Repopulation with exogenous mitochondria by complementation
48. Nuclear complementation restores mtDNA levels in cultured cells from a patient with mtDNA depletion
49. Sequence and organization of the human mitochondrial genome