Mitochondrial DNA and respiratory chain function in spinal cords of ALS patients

Journal of Neurochemistry

Volumn 80, Issue 4, 2002, Pages 616-625

  Wiedemann, Falk R ^a   Manfredi, Giovanni ^a   Mawrin, Christian ^a   Flint Beal, M ^a   Schon, Eric A ^a  

^a OTTO VON GUERICKE UNIVERSITY   (Germany)

Author keywords

Amyotrophic lateral sclerosis; Mitochondrial DNA; Oxidative phosphorylation; Oxygen radicals

Indexed keywords

CITRATE SYNTHASE; CYTOCHROME C OXIDASE; MITOCHONDRIAL DNA; OXYGEN RADICAL;

ADULT; AGED; AMYOTROPHIC LATERAL SCLEROSIS; ARTICLE; CONTROLLED STUDY; DISORDERS OF MITOCHONDRIAL FUNCTIONS; ENZYME ACTIVITY; FEMALE; HUMAN; HUMAN TISSUE; MALE; OXIDATIVE PHOSPHORYLATION; PATHOGENESIS; POINT MUTATION; POLYMERASE CHAIN REACTION; PRIORITY JOURNAL; RESPIRATORY CHAIN; SOUTHERN BLOTTING; SPINAL CORD;

ADULT; AGED; AGED, 80 AND OVER; AMYOTROPHIC LATERAL SCLEROSIS; BIOLOGICAL MARKERS; BLOTTING, SOUTHERN; CHOLINE O-ACETYLTRANSFERASE; CITRATE (SI)-SYNTHASE; DNA MUTATIONAL ANALYSIS; DNA, MITOCHONDRIAL; ELECTRON TRANSPORT; ELECTRON TRANSPORT COMPLEX I; ELECTRON TRANSPORT COMPLEX II; ELECTRON TRANSPORT COMPLEX III; ELECTRON TRANSPORT COMPLEX IV; ENZYME ACTIVATION; FEMALE; HUMANS; MALE; MIDDLE AGED; MULTIENZYME COMPLEXES; NADH, NADPH OXIDOREDUCTASES; OXIDOREDUCTASES; POINT MUTATION; POLYMERASE CHAIN REACTION; SPINAL CORD; SUCCINATE DEHYDROGENASE;

EID: 0036321382     PISSN: 00223042     EISSN: None     Source Type: Journal    
DOI: 10.1046/j.0022-3042.2001.00731.x     Document Type: Article

References (58)

1. Sequence and organization of the human mitochondrial genome
2. Protein oxidative damage in a transgenic mouse model of familial amyotrophic lateral sclerosis
3. Mitochondrial mutations may increase oxidative stress: Implications for carcinogenesis and aging?
4. Aging, energy and oxidative stress in neurodegenerative diseases
5. Mitochondria and the pathogensis of ALS
6. Abnormal RNA processing associated with a novel tRNA mutation in mitochondrial DNA. A potential disease mechanism
7. Increased oxidative damage to DNA in ALS patients
8. Mitochondrial DNA repair pathways
9. Mitochondrial enzyme activity in amyotrophic lateral sclerosis: Implications for the role of mitochondria in neuronal cell death
10. Superoxide dismutase activity, oxidative damage, and mitochondrial energy metabolism in familial and sporadic amyotrophic lateral sclerosis
11. Metabolic dysfunction in familial, but not sporadic, amyotrophic lateral sclerosis
12. Amyotrophic lateral sclerosis: Oxidative energy metabolism and calcium homeostasis in peripheral blood lymphocytes
13. Neurotransmitter-related enzymes in senile dementia of the Alzheimer type
14. Amyotrophic lateral sclerosis and structural defects in Cu, Zn superoxide dismutase
15. Mitochondrial DNA deletion mutation levels are elevated in ALS brains
16. Induction of nitric oxide-dependent apoptosis in motor neurons by zinc-deficient superoxide dismutase
17. Evidence of increased oxidative damage in both sporadic and familial amyotrophic lateral sclerosis
18. Evidence for DNA damage in amyotrophic lateral sclerosis
19. A rapid radiochemical method for the determination of choline acetyltransferase
20. Decreased cytochrome c oxidase activity but unchanged superoxide dismutase and glutathione peroxidase activities in the spinal cords of patients with amyotrophic lateral sclerosis
21. A mutation in the tRNALeu (UUR) gene associated with the MELAS subgroup of mitochondrial encephalomyopathies
22. Nitric oxide-induced damage to mtDNA and its subsequent repair
23. Relationship of oxygen radical-induced lipid peroxidative damage to disease onset and progression in a transgenic model of familial ALS
24. A new mitochondrial disease associated with mitochondrial DNA heteroplasmy
25. Iron, selenium and glutathione peroxidase activity are elevated in sporadic motor neuron disease
26. The nervous tissue
27. Chronic mitochondrial inhibition induces selective motoneuron death in vitro: A new model for amyotrophic lateral sclerosis
28. Human cells lacking mtDNA: Repopulation with exogenous mitochondria by complementation
29. Massive mitochondrial degeneration in motor neurons triggers the onset of amyotrophic lateral sclerosis in mice expressing a mutant SOD1
30. Astrocytes interact intimately with degenerating motor neurons in mouse amyotrophic lateral sclerosis (ALS)
31. The roles of free radicals in amyotrophic lateral sclerosis: Reactive oxygen species and elevated oxidation of protein, DNA, and membrane phospholipids
32. Protein measurement with the Folin phenol reagent
33. Gene-specific repair of gamma-ray-induced DNA strand breaks in colon cancer cells: No coupling to transcription and no removal from the mitochondrial genome
34. An update on the mitochondrial-DNA mutation hypothesis of cell aging
35. Cu/Zn superoxide dismutase free radicals, and motoneuron disease
36. Two novel pathogenic mitochondrial DNA mutations affecting organelle number and protein synthesis. Is the tRNA (Leu (UUR) gene an etiologic hot spot?
37. Hepatic ultrastructural changes and liver dysfunction in amyotrophic lateral sclerosis
38. Immunohistochemical study on choline acetyltransferase in the spinal cord of patients with amyotrophic lateral sclerosis
39. A radical hypothesis for neurodegeneration
40. Evidence that specific mtDNA point mutations may not accumulate in skeletal muscle during normal human aging
41. Protein modification by the lipid peroxidation, product 4-hydroxynonenal in the spinal cords of amyotrophic lateral sclerosis patients
42. Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis
43. Ultrastructural study of synapses in the anterior horn neurons of patients with amyotrophic lateral sclerosis
44. Ultrastructural change of synapses of Betz cells in patients with amyotrophic lateral sclerosis
45. Assays for 8-hydroxy-2′-deoxyguanosine: A biomarker of in vivo oxidative DNA damage
46. Role of uncoupled and non-coupled oxidations in maintenance of safely low levels of oxygen and its one-electron reductants
47. An electron transport system associated with the outer membrane of the mitochondria
48. Mitochondria in sporadic amyotrophic lateral sclerosis
49. Mitochondrial myopathy of childhood associated with depletion of mitochondrial DNA
50. Motor neurons are rich in non-phosphorylated neurofilaments: Cross-species comparison and alterations in ALS
51. Mitochondrial DNA abnormalities in skeletal muscle of patients with sporadic amyotrophic lateral sclerosis
52. Mitochondrial DNA depletion in a patient with long survival
53. Cytochrome oxidase from beef heart mitochondria
54. Impairment of mitochondrial funktion in skeletal muscle of patients with amyotrophic lateral sclerosis
55. m value
56. A gain-of-function of an amyotrophic lateral sclerosis-associated Cu, Zn-superoxide dismutase mutant: An enhancement of free radical formation due to a decrease in Km for hydrogen peroxide
57. Occurrence of a particular base substitution (3243 A to G) in mitochondrial DNA of tissues of ageing humans