Genetic control of oxidative phosphorylation and experimental models of defects

Human Reproduction

Volumn 15, Issue SUPPL. 2, 2000, Pages 18-27

  Trounce, I ^a  

^a ST VINCENT S HOSPITAL   (Australia)

Author keywords

Electron transport chain; Mitochondrial DNA; Oxidative phosphorylation; Respiratory chain; Transmitochondrial cells

Indexed keywords

ADENOSINE DIPHOSPHATE; ADENOSINE TRIPHOSPHATE; CELL NUCLEUS DNA; MITOCHONDRIAL DNA; PROTON TRANSPORTING ADENOSINE TRIPHOSPHATE SYNTHASE;

CELL CULTURE; CELL STRAIN 3T3; CONFERENCE PAPER; DISORDERS OF MITOCHONDRIAL FUNCTIONS; ELECTRON TRANSPORT; EUKARYOTIC CELL; EXPERIMENTAL MODEL; GENETIC ANALYSIS; GENETIC DISORDER; HUMAN; INHERITANCE; KNOCKOUT MOUSE; MITOCHONDRIAL ENERGY TRANSFER; MITOCHONDRION; NONHUMAN; OXIDATIVE PHOSPHORYLATION; PROKARYOTE; RESPIRATORY CHAIN;

BACTERIA (MICROORGANISMS); EUKARYOTA; PROKARYOTA;

EID: 0033647230     PISSN: 02681161     EISSN: None     Source Type: Journal    
DOI: 10.1093/humrep/15.suppl_2.18     Document Type: Conference Paper

References (51)

1. Sequence and organization of the human mitochondrial genome
2. Mitochondrial recombination?
3. Mitochondria, free radicals and neurodegeneration
4. The mitochondrial generation of hydrogen peroxide
5. Complexity and tissue specificity of the mitochondrial respiratory chain
6. Respiratory enzymes in oxidative phosphorylation I-IV
7. In vitro genetic transfer of protein sythesis and respiration defects to mitochondrial DNA-less cells with myopathy-patient mitochondria
8. Genetic defects causing human mitochondrial respiratory chain disorders and disease
9. Transcription and replication of mitochondrial DNA
10. Cytoplasmic DNA
11. Nuclear power and mitochondrial disease
12. The interplay of heredity and environment in the synthesis of respiratory enzymes in yeast
13. How to find all those mutations
14. Maternal inheritance of human mitochondrial DNA
15. A mouse model for mitochondrial myopathy and cardiomyopathy resulting from a deficiency in the heart/muscle isoform of the adenine nucleotide translocator
16. Mitochondrial evolution
17. Evolutionary origin and consequences of uniparental mitochondrial inheritance
18. Mitochondrial chloramphenicol-resistant mutants can have deficiencies in energy metabolism
19. Isolation and microinjection of somatic cell-derived mitochondria and germline heteroplasmy in mice
20. Origin and persistence of the mitochondrial genome
21. Random genetic drift in the female germline explains the rapid segregation of mammalian mitochondrial DNA
22. Expanding the functional human mitochondrial DNA database by the establishment of primate xenomitochondrial cybrids
23. Human cells lacking mtDNA: Repopulation with exogenous mitochondria by complementation
24. Defects in mitochondrial protein synthesis and respiratory chain activity segregate with the tRNA Leu mutation associated with MELAS
25. The release of cytochrome c from mitochondria: A primary site for Bcl-2 regulation of apoptosis
26. Substrate and site specificity of hydrogen peroxide generation in mouse mitochondria
27. The plant mitochondrial genome and its mutants
28. Infertility treatment: A nuclear restorer gene in Maize
29. Transfer of chloramphenicol-resistant mitochondrial DNA into the chimeric mouse
30. Mammalian mitochondrial genetics: Heredity, heteroplasmy and disease
31. Mitochondrial DNA: All things bad?
32. Key to the mitochondrial gate
33. The mitochondrial F0F1-ATPase proton pump is required for function of the proapoptotic protein Bax in yeast and mammalian cells
34. Mitochondrial disease in superoxide dismutase 2 mutant mice
35. Mitochondrial genotype segregation during pre-implantation development in mouse heteroplasmic embryos
36. Development and characterization of continuous avian cell lines depleted of mitochondrial DNA
37. Organismal effects of mitochondrial dysfunction
38. Cell-free apoptosis in Xenopus egg extracts: Inhibition by Bcl-2 and requirement for an organelle fraction enriched in mitochondria
39. Cell suicide for beginners
40. Regulation of mitochondrial DNA copy number during spermatogenesis
41. Replication and inheritance of mitochondrial DNA
42. Oxidative phosphorylation at the fin de siecle
43. Production of transmitochondrial mouse cell lines by cybrid rescue of rhodamine-6G pre-treated L cells
44. Cytoplasmic transfer of the mtDNA nt8993 T-G (ATP6) point mutation associated with Leigh syndrome into mtDNA-less cells demonstrates cosegregation with a decrease in state III respiration and ADP/O ratio
45. Assessment of mitochondrial oxidative phosphorylation in patient muscle biopsies, lymphoblasts, and transmitochondrial cell lines
46. Mitochondrial diseases in man and mouse
47. The creation of mouse models for mitochondrial DNA (mtDNA) disease
48. Dilated cardiomyopathy and atrioventricular conduction blocks induced by heart-specific inactivation of mitochondrial DNA gene expression
49. Prevention of apoptosis by Bcl-2: Release of cytochrome c from mitochondria blocked