Citrulline and arginine utility in treating nitric oxide deficiency in mitochondrial disorders

Molecular Genetics and Metabolism

Volumn 107, Issue 3, 2012, Pages 247-252

  El Hattab, Ayman W ^a   Emrick, Lisa T ^b   Craigen, William J ^b   Scaglia, Fernando ^b  

^a KING SAUD BIN ABDULAZIZ UNIVERSITY FOR HEALTH SCIENCES   (Saudi Arabia)

^b BAYLOR COLLEGE OF MEDICINE   (United States)

Author keywords

Endothelial dysfunction; Lactic acidosis; Myopathy; Nitric oxide deficiency; Nitric oxide synthase (NOS); Stroke like episodes

Indexed keywords

ADENOSINE TRIPHOSPHATE; ARGININE; ARGININOSUCCINATE LYASE; ARGININOSUCCINATE SYNTHASE; CITRULLINE; CYTOCHROME C OXIDASE; DIMETHYLARGININASE; NITRIC OXIDE; NITRIC OXIDE SYNTHASE;

AMINO ACID METABOLISM; CEREBROVASCULAR ACCIDENT; CLINICAL FEATURE; DIABETES MELLITUS; DISORDERS OF MITOCHONDRIAL FUNCTIONS; DRUG ABSORPTION; DRUG BIOAVAILABILITY; DRUG EFFICACY; DRUG SCREENING; LACTIC ACIDOSIS; MAXIMUM PLASMA CONCENTRATION; MEDICAL RESEARCH; MELAS SYNDROME; METABOLIC DISORDER; MYOPATHY; NIRTRIC OXIDE DEFICIENCY; NITRIC OXIDE DEFICIENCY; PATHOGENESIS; PRIORITY JOURNAL; RESPIRATORY CHAIN; REVIEW; SUPPLEMENTATION; TREATMENT RESPONSE;

ARGININE; ARGININOSUCCINATE LYASE; ARGININOSUCCINATE SYNTHASE; CITRULLINE; CLINICAL TRIALS AS TOPIC; ELECTRON TRANSPORT; ELECTRON TRANSPORT COMPLEX IV; HUMANS; MELAS SYNDROME; MITOCHONDRIA; NITRIC OXIDE; NITRIC OXIDE SYNTHASE TYPE III; REACTIVE NITROGEN SPECIES;

EID: 84867895466     PISSN: 10967192     EISSN: 10967206     Source Type: Journal    
DOI: 10.1016/j.ymgme.2012.06.018     Document Type: Review

References (49)

1. Wallace D.C. Mitochondrial diseases in man and mouse. Science 1999, 283:1482-1488.
2. Tengan C.H., Kiyomoto B.H., Godinho R.O., et al. The role of nitric oxide in muscle fibers with oxidative phosphorylation defects. Biochem. Biophys. Res. Commun. 2007, 359:771-777.
3. Koga Y., Akita Y., Nishioka J., et al. L-Arginine improves the symptoms of strokelike episodes in MELAS. Neurology 2005, 64:710-712.
4. Koga Y., Akita Y., Junko N., et al. Endothelial dysfunction in MELAS improved by l-arginine supplementation. Neurology 2006, 66:1766-1769.
5. Koga Y., Akita Y., Nishioka J., et al. MELAS and l-arginine therapy. Mitochondrion 2007, 7:133-139.
6. Vattemi G., Mechref Y., Marini M., et al. Increased protein nitration in mitochondrial diseases: evidence for vessel wall involvement. Mol. Cell. Proteomics 2011, 10. (M110.002964).
7. El-Hattab A.W., Hsu J.W., Emrick L.T., et al. Restoration of impaired nitric oxide production in MELAS syndrome with citrulline and arginine supplementation. Mol. Genet. Metab. 2012, 105:607-614.
8. Wu G., Morris S.M. Arginine metabolism: nitric oxide and beyond. Biochem. J. 1998, 336:1-17.
9. Barbul A. Arginine: biochemistry, physiology, and therapeutic implications. JPEN J. Parenter. Enteral. Nutr. 1986, 10:227-238.
10. Flynn N.E., Meininger C.J., Haynes T.E., Wu G. The metabolic basis of arginine nutrition and pharmacotherapy. Biomed. Pharmacother. 2002, 56:427-438.
11. Morris S.M. Arginine metabolism: boundaries of our knowledge. J. Nutr. 2007, 137:1602S-1609S.
12. Curis E., Nicolis I., Moinard C., et al. Almost all about citrulline in mammals. Amino Acids 2005, 29:177-205.
13. Moinard C., Cynober L. Citrulline: a new player in the control of nitrogen homeostasis. J. Nutr. 2007, 137:1621S-1625S.
14. Wu G. Intestinal mucosal amino acid catabolism. J. Nutr. 1998, 128:1249-1252.
15. Jackson M.J., Beaudet A.L., O'Brien W.E. Mammalian urea cycle enzymes. Annu. Rev. Genet. 1986, 20:431-464.
16. Morris S.M. Regulation of enzymes of urea and arginine synthesis. Annu. Rev. Nutr. 1992, 12:81-101.
17. Husson A., Brasse-Lagnel C., Fairand A., Renouf S., Lavoinne A. Argininosuccinate synthetase from the urea cycle to the citrulline-NO cycle. Eur. J. Biochem. 2003, 270:1887-1899.
18. Windmueller H.G., Spaeth A.E. Source and fate of circulating citrulline. Am. J. Physiol. 1981, 241:E473-E480.
19. Dhanakoti S.N., Brosnan J.T., Herzberg G.R., Brosnan M.E. Renal arginine synthesis: studies in vitro and in vivo. Am. J. Physiol. 1990, 259:E437-E442.
20. Förstermann U., Closs E.I., Pollock J.S., et al. Nitric oxide synthase isozymes. Characterization, purification, molecular cloning, and functions. Hypertension 1994, 23:1121-1131.
21. Villanueva C., Giulivi C. Subcellular and cellular locations of nitric oxide synthase isoforms as determinants of health and disease. Free Radic. Biol. Med. 2010, 49:307-316.
22. Mori M., Gotoh T. Regulation of nitric oxide production by arginine metabolic enzymes. Biochem. Biophys. Res. Commun. 2000, 3:715-719.
23. Oyadomari S., Gotoh T., Aoyagi K., Araki E., Shichiri M., Mori M. Coinduction of endothelial nitric oxide synthase and arginine recycling enzymes in aorta of diabetic rats. Nitric Oxide 2001, 5:252-260.
24. Flam B.R., Hartmann P.J., Harrell-Booth M., Solomonson L.P., Eichler D.C. Caveolar localization of arginine regeneration enzymes, argininosuccinate synthase, and lyase, with endothelial nitric oxide synthase. Nitric Oxide 2001, 5:187-197.
25. Solomonson L.P., Flam B.R., Pendleton L.C., Goodwin B.L., Eichler D.C. The caveolar nitric oxide synthase/arginine regeneration system for NO production in endothelial cells. J. Exp. Biol. 2003, 206:2083-2087.
26. Erez A., Nagamani S.C., Shchelochkov O.A., et al. Requirement of argininosuccinate lyase for systemic nitric oxide production. Nat. Med. 2011, 17:1619-1626.
27. Ohama E., Ohara S., Ikuta F., et al. Mitochondrial angiopathy in cerebral blood vessels of mitochondrial encephalomyopathy. Acta Neuropathol. 1987, 74:226-233.
28. Goto Y., Horai S., Matsuoka T., et al. Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS): a correlative study of the clinical features and mitochondrial DNA mutation. Neurology 1992, 42:545-550.
29. Toda N., Okamura T. The pharmacology of nitric oxide in the peripheral nervous system of blood vessels. Pharmacol. Rev. 2003, 55:271-324.
30. Green D.J., Maiorana A., O'Driscoll G., Taylor R. Effect of exercise training on endothelium-derived nitric oxide function in humans. J. Physiol. 2004, 561:1-25.
31. Naini A., Kaufmann P., Shanske S., et al. Hypocitrullinemia in patients with MELAS: an insight into the "MELAS paradox". J. Neurol. Sci. 2005, 229:87-193.
32. Sproule D.M., Kaufmann P. Mitochondrial encephalopathy, lactic acidosis, and strokelike episodes: basic concepts, clinical phenotype, and therapeutic management of MELAS syndrome. Ann. N. Y. Acad. Sci. 2008, 1142:133-158.
33. Wallace D.C., Brown M.D., Melov S., Graham B., Lott M. Mitochondrial biology, degenerative diseases and aging. Biofactors 1998, 7:187-190.
34. Parfait B., de Lonlay P., von Kleist-Retzow J.C., et al. The neurogenic weakness, ataxia and retinitis pigmentosa (NARP) syndrome mtDNA mutation (T8993G) triggers muscle ATPase deficiency and hypocitrullinaemia. Eur. J. Pediatr. 1999, 55:55-58.
35. Castillo L., Chapman T.E., Sanchez M., et al. Plasma arginine and citrulline kinetics in adults given adequate and arginine-free diets. Proc. Natl. Acad. Sci. U. S. A. 1993, 90:7749-7753.
36. Teerlink T., Luo Z., Palm F., Wilcox C.S. Cellular ADMA: regulation and action. Pharmacol. Res. 2009, 60:448-460.
37. Bode-Böger S.M., Böger R.H., Schröder E.P., Frölich J.C. Exercise increases systemic nitric oxide production in men. J. Cardiovasc. Risk 1994, 1:173-178.
38. Schrage W.G., Joyner M.J., Dinenno F.A. Local inhibition of nitric oxide and prostaglandins independently reduces forearm exercise hyperaemia in humans. J. Physiol. 2004, 557:599-611.
39. Maassen J.A., Hart L.M'.t, Van Essen E., et al. Mitochondrial diabetes: molecular mechanisms and clinical presentation. Diabetes 2004, 53:S103-S109.
40. Jonk A.M., Houben A.J., de Jongh R.T., Serne E.H., Schaper N.C., Stehouwer C.D. Microvascular dysfunction in obesity: a potential mechanism in the pathogenesis of obesity-associated insulin resistance and hypertension. Physiology (Bethesda) 2007, 22:252-260.
41. Serne E.H., de Jongh R.T., Eringa E.C., Ijzerman R.G., Stehouwer C.D. Microvascular dysfunction: a potential pathophysiological role in the metabolic syndrome. Hypertension 2007, 50:204-211.
42. Baron A.D. Cardiovascular actions of insulin in humans. Implications for insulin sensitivity and vascular tone, Baillieres. Clin. Endocrinol. Metab. 1993, 7:961-987.
43. Baron A.D. Hemodynamic actions of insulin. Am. J. Physiol. Endocrinol. Metab. 1994, 267:E187-E202.
44. Kim J.A., Montagnani M., Koh K.K., Quon M.J. Reciprocal relationships between insulin resistance and endothelial dysfunction: molecular and pathophysiological mechanisms. Circulation 2006, 13:1888-1904.
45. Potenza M.A., Marasciulo F.L., Chieppa D.M., et al. Insulin resistance in spontaneously hypertensive rats is associated with endothelial dysfunction characterized by imbalance between NO and ET-1 production. Am. J. Physiol. Heart Circ. Physiol. 2005, 289:H813-H822.
46. Coman D., Yaplito-Lee J., Boneh A. New indications and controversies in arginine therapy. Clin. Nutr. 2008, 27:489-496.
47. Cynober L. Pharmacokinetics of arginine and related amino acids. J. Nutr. 2007, 137:1646S-1649S.
48. Moinard C., Nicolis I., Neveux N., Darquy S., Bénazeth S., Cynober L. Dose-ranging effects of citrulline administration on plasma amino acids and hormonal patterns in healthy subjects: the Citrudose pharmacokinetic study. Br. J. Nutr. 2008, 99:855-862.
49. Schwedhelm E., Maas R., Freese R., et al. Pharmacokinetic and pharmacodynamic properties of oral l-citrulline and l-arginine: impact on nitric oxide metabolism. Br. J. Clin. Pharmacol. 2008, 65:51-59.