A Mouse Model of Familial ALS Has Increased CNS Levels of Endogenous Ubiquinol9/10 and Does Not Benefit from Exogenous Administration of Ubiquinol10

PLoS ONE

Volumn 8, Issue 7, 2013, Pages

  Lucchetti, Jacopo ^a   Marino, Marianna ^a   Papa, Simonetta ^a   Tortarolo, Massimo ^a   Guiso, Giovanna ^a   Pozzi, Silvia ^a   Bonetto, Valentina ^a   Caccia, Silvio ^a   Beghi, Ettore ^a   Bendotti, Caterina ^a   Gobbi, Marco ^a  

^a MARIO NEGRI INSTITUTE FOR PHARMACOLOGICAL RESEARCH   (Italy)

Author keywords

[No Author keywords available]

Indexed keywords

KANEKA QH; QUINONE DERIVATIVE; UBIDECARENONE; UBIQUINOL 10; UBIQUINOL 9; UBIQUINONE 9; UNCLASSIFIED DRUG;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BLOOD LEVEL; BRAIN LEVEL; CONTROLLED STUDY; DISEASE COURSE; DRUG EFFECT; FAMILIAL DISEASE; FEMALE; LONG TERM CARE; LUMBAR SPINAL CORD; MALE; MOUSE; NONHUMAN; OXIDATION REDUCTION REACTION; SINGLE DRUG DOSE; SURVIVAL;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMALS; BRAIN; CENTRAL NERVOUS SYSTEM; DISEASE MODELS, ANIMAL; DISEASE PROGRESSION; HUMANS; MICE; MICE, TRANSGENIC; SPINAL CORD; SUPEROXIDE DISMUTASE; UBIQUINONE;

EID: 84880716238     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0069540     Document Type: Article

References (29)

1. Tran UC, Clarke CF, (2007) Endogenous synthesis of coenzyme Q in eukaryotes. Mitochondrion 7Suppl: S62-71.
2. Lass A, Agarwal S, Sohal RS, (1997) Mitochondrial ubiquinone homologues, superoxide radical generation, and longevity in different mammalian species. J Biol Chem 272: 19199-19204.
3. Tang PH, Miles MV, Miles L, Quinlan J, Wong B, et al. (2004) Measurement of reduced and oxidized coenzyme Q9 and coenzyme Q10 levels in mouse tissues by HPLC with coulometric detection. Clin Chim Acta 341: 173-184.
4. Littarru GP, Langsjoen P, (2007) Coenzyme Q10 and statins: biochemical and clinical implications. Mitochondrion 7Suppl: S168-174.
5. Pravst I, Zmitek K, Zmitek J, (2010) Coenzyme Q10 contents in foods and fortification strategies. Crit Rev Food Sci Nutr 50: 269-280.
6. Huertas JR, Battino M, Lenaz G, Mataix FJ, (1991) Changes in mitochondrial and microsomal rat liver coenzyme Q9 and Q10 content induced by dietary fat and endogenous lipid peroxidation. FEBS Lett 287: 89-92.
7. Edlund C, Soderberg M, Kristensson K, (1994) Isoprenoids in aging and neurodegeneration. Neurochem Int 25: 35-38.
8. Lagendijk J, Ubbink JB, Vermaak WJ, (1996) Measurement of the ratio between the reduced and oxidized forms of coenzyme Q10 in human plasma as a possible marker of oxidative stress. J Lipid Res 37: 67-75.
9. Sohmiya M, Tanaka M, Suzuki Y, Tanino Y, Okamoto K, et al. (2005) An increase of oxidized coenzyme Q-10 occurs in the plasma of sporadic ALS patients. J Neurol Sci 228: 49-53.
10. Murata T, Ohtsuka C, Terayama Y, (2008) Increased mitochondrial oxidative damage in patients with sporadic amyotrophic lateral sclerosis. J Neurol Sci 267: 66-69.
11. Bendotti C, Carri MT, (2004) Lessons from models of SOD1-linked familial ALS. Trends Mol Med 10: 393-400.
12. Matthews RT, Yang L, Browne S, Baik M, Beal MF, (1998) Coenzyme Q10 administration increases brain mitochondrial concentrations and exerts neuroprotective effects. Proc Natl Acad Sci U S A 95: 8892-8897.
13. Kaufmann P, Thompson JL, Levy G, Buchsbaum R, Shefner J, et al. (2009) Phase II trial of CoQ10 for ALS finds insufficient evidence to justify phase III. Ann Neurol 66: 235-244.
14. Hosoe K, Kitano M, Kishida H, Kubo H, Fujii K, et al. (2007) Study on safety and bioavailability of ubiquinol (Kaneka QH) after single and 4-week multiple oral administration to healthy volunteers. Regul Toxicol Pharmacol 47: 19-28.
15. Kitano M, Watanabe D, Oda S, Kubo H, Kishida H, et al. (2008) Subchronic oral toxicity of ubiquinol in rats and dogs. Int J Toxicol 27: 189-215.
16. Cleren C, Yang L, Lorenzo B, Calingasan NY, Schomer A, et al. (2008) Therapeutic effects of coenzyme Q10 (CoQ10) and reduced CoQ10 in the MPTP model of Parkinsonism. J Neurochem 104: 1613-1621.
17. Pizzasegola C, Caron I, Daleno C, Ronchi A, Minoia C, et al. (2009) Treatment with lithium carbonate does not improve disease progression in two different strains of SOD1 mutant mice. Amyotroph Lateral Scler 10: 221-228.
18. Mosca F, Fattorini D, Bompadre S, Littarru GP, (2002) Assay of coenzyme Q(10) in plasma by a single dilution step. Anal Biochem 305: 49-54.
19. Takada M, Ikenoya S, Yuzuriha T, Katayama K, (1984) Simultaneous determination of reduced and oxidized ubiquinones. Methods Enzymol 105: 147-155.
20. Podda M, Weber C, Traber MG, Packer L, (1996) Simultaneous determination of tissue tocopherols, tocotrienols, ubiquinols, and ubiquinones. J Lipid Res 37: 893-901.
21. Takada M, Ikenoya S, Yuzuriha T, Katayama K, (1982) Studies on reduced and oxidized coenzyme Q (ubiquinones). II. The determination of oxidation-reduction levels of coenzyme Q in mitochondria, microsomes and plasma by high-performance liquid chromatography. Biochim Biophys Acta 679: 308-314.
22. Bentinger M, Brismar K, Dallner G, (2007) The antioxidant role of coenzyme Q. Mitochondrion. 7Suppl: S41-50.
23. Ludolph AC, Bendotti C, Blaugrund E, Chio A, Greensmith L, et al. (2010) Guidelines for preclinical animal research in ALS/MND: A consensus meeting. Amyotroph Lateral Scler 11: 38-45.
24. Poderoso JJ, (2009) The formation of peroxynitrite in the applied physiology of mitochondrial nitric oxide. Arch Biochem Biophys 484: 214-220.
25. Schopfer F, Riobo N, Carreras MC, Alvarez B, Radi R, et al. (2000) Oxidation of ubiquinol by peroxynitrite: implications for protection of mitochondria against nitrosative damage. Biochem J 349: 35-42.
26. Casoni F, Basso M, Massignan T, Gianazza E, Cheroni C, et al. (2005) Protein nitration in a mouse model of familial amyotrophic lateral sclerosis: possible multifunctional role in the pathogenesis. J Biol Chem 280: 16295-16304.
27. Basso M, Samengo G, Nardo G, Massignan T, D'Alessandro G, et al. (2009) Characterization of detergent-insoluble proteins in ALS indicates a causal link between nitrative stress and aggregation in pathogenesis. PLoS One 4: e8130.
28. Kontush A, Schippling S, Spranger T, Beisiegel U, (1999) Plasma ubiquinol-10 as a marker for disease: is the assay worthwhile? Biofactors 9: 225-229.
29. Edlund C, Soderberg M, Kristensson K, Dallner G, (1992) Ubiquinone, dolichol, and cholesterol metabolism in aging and Alzheimer's disease. Biochem Cell Biol 70: 422-428.