Progress toward acetate supplementation therapy for Canavan disease: Glyceryl triacetate administration increases acetate, but not N-acetylaspartate, levels in brain

Journal of Pharmacology and Experimental Therapeutics

Volumn 315, Issue 1, 2005, Pages 297-303

  Mathew, Raji ^a   Arun, Peethambaran ^a   Madhavarao, Chikkathur N ^a   Moffett, John R ^a   Namboodiri, M A Aryan ^a  

^a UNIFORMED SERVICES UNIVERSITY OF THE HEALTH SCIENCES   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ACETIC ACID; ACYLTRANSFERASE; TRIACETIN;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CANAVAN DISEASE; CONTROLLED STUDY; ENZYME LOCALIZATION; GENE MUTATION; MOUSE; NONHUMAN; OLIGODENDROGLIA; PRIORITY JOURNAL;

ACETATES; ANIMALS; ASPARTIC ACID; BRAIN; CANAVAN DISEASE; LIVER; MALE; MICE; MICE, INBRED C57BL;

EID: 25644446862     PISSN: 00223565     EISSN: None     Source Type: Journal    
DOI: 10.1124/jpet.105.087536     Document Type: Article

References (42)

1. Adachi M, Schneck L, Cara J, and Volk BW (1973) Spongy degeneration of the central nervous system. Hum Pathol 4:331-347.
2. Bach A and Metais P (1970) Graisses à chaînes nes courtes et moyennes: aspects physiologiques, biochimiques, nutritionnels et thérapeutiques. Ann Nutr Alim 24:75-144.
3. Bailey JW, Haymond MW, and Miles JM (1991) GTA: a potential parenteral nutrient. J Parenter Enteral Nutr 15:32-36.
4. Bailey JW, Heath H, and Miles JM (1989) Calcium, magnesium and phosphorous metabolism in dogs given intravenous GTA. Am J Clin Nutr 49:385-388.
5. Bailey JW, Miles JM, and Haymond MW (1993) Effect of parenteral administration of short chain triglycerides on leucine metabolism. Am J Clin Nutr 58:912-916.
6. Baslow MH (2003) Brain N-acetylaspartate as a molecular water pump and its role in the etiology of Canavan disease: a mechanistic explanation. J Mol Neurosci 21:185-190.
7. Burri R, Steffen C, and Herschkowitz N (1991) N-Acetyl-L-aspartate is a major source of acetyl groups for lipid synthesis during rat brain development. Dev Neurosci 13:403-411.
8. Chakraborty G, Mekala P, Yahya D, Wu G, and Ledeen RW (2001) Intraneuronal N-acetylaspartate supplies acetyl groups for myelin lipid synthesis: evidence for myelin-associated aspartoacylase. J Neurochem 78:736-745.
9. +. Neurochem Res 12:431-438.
10. D'adamo AF Jr, Gidez LI, and Yatsu FM (1968) Acetyl transport mechanisms: involvement of N-acetylaspartic acid in de novo fatty acid biosynthesis in the developing rat brain. Exp Brain Res 5:267-273.
11. D'adamo AF Jr and Yatsu FM (1966) Acetate metabolism in the nervous system: N-acetylaspartic acid and the biosynthesis of brain lipids. J Neurochem 13:961-965.
12. Dhopeshwarker GA and Mead JF (1973) Uptake and transport of fatty acids into the brain and the role of blood-brain barrier system. Adv Lipid Res 11:109-142.
13. Durst D, Milano M, Kikta EJ, Connelly SA, and Grushka E (1975) Phenacyl esters of fatty acids via crown ether catalysts for enhanced ultraviolet detection in liquid chromatography. Anal Chem 47:1797-1801.
14. Fiume MZ (2003) Final report on the safety assessment of Triacetin. Int J Toxicol 22 (Suppl 2):1-10.
15. Florian CL, Williams SR, Bhakoo KK, and Noble MD (1996) Regional and developmental variations in metabolite concentrations in the rat brain and eye: a study using 1H NMR spectroscopy and high performance liquid chromatography. Neurochem Res 9:1065-1074.
16. Hagenfeldt L, Bollgren I, and Venizelos N (1987) N-Acetylated aciduria due to aspartoacylase deficiency-a new etiology of childhood leukodystrophy. J Inher Metab Dis 10:135-141.
17. Kamoshita S, Rapin I, and Suzuki K (1968) Spongy degeneration of the brain: a chemical study of two cases including isolation and characterization of myelin. Neurology 18:975-986.
18. Kaul R, Gao GP, Aloya M, Balamurugan K, Petrosky A, Michals K, and Matalon R (1994) Canavan disease: mutations among Jewish and non-Jewish patients. Am J Hum Genet 55:34-41.
19. Kirmani BF, Jacobowitz DM, Kallarakal AT, and Namboodiri MAA (2002) Aspartoacylase is restricted primarily to myelin synthesizing cells in the CNS: therapeutic implications for Canavan disease. Brain Res Mol Brain Res 107:176-182.
20. Kiselevski Y, Oganesian N, Zimatkin S, Szutowicz A, Angielski S, Niezabitowski P, Uracz W, and Gryglewski R (2003) Acetate metabolism in brain mechanisms of adaptation to ethanol. Med Sci Monit 9:BR218-BR222.
21. Klugmann M, Leichtlein CB, Symes CW, Serikawa T, Young D, and During AJ (2005) Restoration of aspartoacylase activity in CNS neurons does not ameliorate motor deficits and demyelination in a model of Canavan disease. Mol Ther 11:745-753.
22. Koller KJ and Coyle JT (1984) Ontogenics of N-acetyl-aspartate and N-acetyl-aspartyl-glutamate in rat brain. Brain Res 317:137-140.
23. Kvittingen EA, Guldal G, Borsting S, Skalpe IO, Stokke O, and Jellum E (1986) N-Acetylated aciduria in a child with a progressive cerebral atrophy. Clin Chim Acta 158:217-227.
24. Madhavarao CN, Arun P, Moffett JR, Szucs S, Surendran S, Matalon R, Garbern J, Hristova D, Johnson A, Jiang W, et al. (2005) Defective N-acetylaspartate catabolism reduces brain acetate levels and myelin lipid synthesis in Canavan's disease. Proc Natl Acad Sci USA 102:5221-5226.
25. Madhavarao CN, Chinopoulos C, Chandrasekaran K, and Namboodiri MAA (2003) Characterization of the N-acetylaspartate biosynthetic enzyme from rat brain. J Neurochem 86:824-835.
26. Madhavarao CN, Hammer JA, Quarles RH, and Namboodiri MAA (2002) A radiometric assay for aspartoacylase activity in cultured oligodendrocytes. Anal Biochem 308:314-319.
27. Madhavarao CN, Moffett JR, Moore RA, Viola RE, Namboodiri MAA, and Jacobowitz DM (2004) Immunohistochemical localization of aspartoacylase in the rat central nervous system. J Comp Neurol 472:318-329.
28. Matalon R, Michals K, and Kaul R (1995) Canavan disease: from spongy degeneration to molecular analysis. J Pediatr 127:511-517.
29. Matalon R, Michals K, Sebasta D, Deaching M, Gashkoff P, and Casanova J (1988) Aspartoacylase deficiency and N-acetylaspartic adiduria in patients with Canavan disease. Am J Med Genet 29:463-471.
30. Mehta V and Namboodiri MAA (1995) N-Acetylaspartate as an acetyl source in the nervous system. Mol Brain Res 31:151-157.
31. Moffett JR and Namboodiri MA (1995) Differential distribution of N-acetylaspartylglutamate and N-acetylaspartate immunoreactivities in rat forebrain. J Neurocytol 24:409-433.
32. 13C] glucose infusion J Neurochem 77:347-350.
33. Namboodiri MAA and Klein DC (1979) Use of charcoal adsorption for the microassay of acetyl CoA hydrolase. Anal Biochem 93:244-247.
34. Namboodiri MAA, Murphy AC, Jiang G, Rollag M, and Provenio I (2000) Murine aspartoacylase: cloning, expression and comparison with the human enzyme. Mol Brain Res 77:285-289.
35. Patience RL and Thomas JD (1982) Rapid concentration and analysis of short chain carboxylic acids: variation on a theme. J Chromatogr 234:225-230.
36. Persson H, Bleiberg B, Kiss D, and Miles J (1991) Measurement of plasma acetate kinetics using high-performance liquid chromatography. J Biochem 198:149-153.
37. Schiller LR, Santa Ana CA, and Sheikh MS (1989) Effect of the time of administration of calcium acetate on phosphorous binding. N Engl J Med 320:1110-1113.
38. Tallan HH (1957) Studies on the distribution of N-acetyl-L-aspartic acid in brain. Biol Chem 224:41-45.
39. Tallan HH, Moore S, and Stein WH (1956) N-Acetyl-L-aspartic acid in brain. J Biol Chem 219:257-264.
40. Terasaki T, Takakuwa S, Moritani S, and Tsuji A (1991) Transport of monocarboxylic acids at the blood-brain barrier: studies with monolayers of primary cultured bovine brain capillary endothelial cells. J Pharmacol Exp Ther 258:932-937.
41. Waniewski RA and Martin DL (1998) Preferential utilization of acetate by astrocytes is attributable to transport. J Neurosci 18:5225-5233.
42. Wittsack HJ, Kugel H, Roth B, and Heindel W (1996) Quantitative measurements with localized proton NMR spectroscopy in children with Canavan disease. J Magn Reson Imaging 6:889-893.