title: Role of carnitine in disease.
abstract: Carnitine is a conditionally essential nutrient that plays a vital role in energy production and fatty acid metabolism. Vegetarians possess a greater bioavailability than meat eaters. Distinct deficiencies arise either from genetic mutation of carnitine transporters or in association with other disorders such as liver or kidney disease. Carnitine deficiency occurs in aberrations of carnitine regulation in disorders such as diabetes, sepsis, cardiomyopathy, malnutrition, cirrhosis, endocrine disorders and with aging. Nutritional supplementation of L-carnitine, the biologically active form of carnitine, is ameliorative for uremic patients, and can improve nerve conduction, neuropathic pain and immune function in diabetes patients while it is life-saving for patients suffering primary carnitine deficiency. Clinical application of carnitine holds much promise in a range of neural disorders such as Alzheimer's disease, hepatic encephalopathy and other painful neuropathies. Topical application in dry eye offers osmoprotection and modulates immune and inflammatory responses. Carnitine has been recognized as a nutritional supplement in cardiovascular disease and there is increasing evidence that carnitine supplementation may be beneficial in treating obesity, improving glucose intolerance and total energy expenditure.
authors.affiliation: Institute for Eye Research, Sydney, New South Wales, Australia. j.flanagan@ier.org.au.
authors.forename: Judith L
authors.initials: JL
authors.lastname: Flanagan
authors.forename: Peter A
authors.initials: PA
authors.lastname: Simmons
authors.forename: Joseph
authors.initials: J
authors.lastname: Vehige
authors.forename: Mark Dp
authors.initials: MD
authors.lastname: Willcox
authors.forename: Qian
authors.initials: Q
authors.lastname: Garrett
references.citation: Ann N Y Acad Sci. 2004 Nov;1033:30-41
references.pmid: 15591001
references.citation: J Inherit Metab Dis. 2003;26(6):613-5
references.pmid: 14605509
references.citation: Epilepsia. 1994 Sep-Oct;35(5):1005-15
references.pmid: 7925143
references.citation: Clin Nephrol. 2002 May;57(5):402-5
references.pmid: 12036203
references.citation: Nephron. 1988;50(2):89-102
references.pmid: 3065666
references.citation: Am J Dis Child. 1987 Jun;141(6):660-5
references.pmid: 3578191
references.citation: Biochem Genet. 2008 Feb;46(1-2):54-63
references.pmid: 17987377
references.citation: Exp Eye Res. 1997 Feb;64(2):195-201
references.pmid: 9176053
references.citation: Arch Dis Child. 2000 Jan;82(1):67-70
references.pmid: 10630918
references.citation: Neurology. 2002 Oct 8;59(7):1046-51
references.pmid: 12370460
references.citation: Diabetes. 2002 Feb;51(2):271-5
references.pmid: 11812732
references.citation: Mol Neurobiol. 2008 Oct;38(2):146-52
references.pmid: 18726077
references.citation: J Physiol Pharmacol. 2008 Dec;59 Suppl 7:19-30
references.pmid: 19258655
references.citation: Curr Eye Res. 1998 Jun;17(6):551-9
references.pmid: 9663844
references.citation: Neurogenetics. 2005 Sep;6(3):107-26
references.pmid: 15965709
references.citation: Hum Mol Genet. 1999 Apr;8(4):655-60
references.pmid: 10072434
references.citation: J Nutr. 1968 Dec;96(4):499-504
references.pmid: 5699991
references.citation: Muscle Nerve. 2001 Feb;24(2):170-91
references.pmid: 11180201
references.citation: New Horiz. 1994 Nov;2(4):505-11
references.pmid: 7804799
references.citation: Annu Rev Nutr. 1998;18:39-61
references.pmid: 9706218
references.citation: FEBS Lett. 1997 Jun 16;409(3):401-6
references.pmid: 9224698
references.citation: Biochem J. 2002 Feb 1;361(Pt 3):417-29
references.pmid: 11802770
references.citation: N Engl J Med. 1988 Sep 1;319(9):548-55
references.pmid: 3136331
references.citation: Curr Opin Pediatr. 1998 Dec;10(6):615-21
references.pmid: 9848022
references.citation: Mol Genet Metab. 2002 Nov;77(3):195-201
references.pmid: 12409266
references.citation: Hepatology. 1997 Mar;25(3):551-6
references.pmid: 9049197
references.citation: Postgrad Med J. 1996 Jan;72(843):45-50
references.pmid: 8746285
references.citation: Dig Dis Sci. 2008 Nov;53(11):3018-25
references.pmid: 18357530
references.citation: J Clin Invest. 1977 Sep;60(3):716-23
references.pmid: 893675
references.citation: Biochem Soc Trans. 2003 Dec;31(Pt 6):1133-6
references.pmid: 14641011
references.citation: Biochem Med. 1975 Jan;12(1):12-21
references.pmid: 1137568
references.citation: Metabolism. 1985 Aug;34(8):721-5
references.pmid: 3927109
references.citation: Cornea. 2008 Jun;27(5):574-9
references.pmid: 18520508
references.citation: J Am Coll Cardiol. 1995 Aug;26(2):380-7
references.pmid: 7608438
references.citation: JPEN J Parenter Enteral Nutr. 1985 Jul-Aug;9(4):483-90
references.pmid: 3928925
references.citation: Optom Vis Sci. 2009 Feb;86(2):E132-8
references.pmid: 19156017
references.citation: J Physiol Biochem. 2008 Mar;64(1):1-8
references.pmid: 18663990
references.citation: Mol Psychiatry. 2000 Nov;5(6):616-32
references.pmid: 11126392
references.citation: Invest Ophthalmol Vis Sci. 2007 Jun;48(6):2553-60
references.pmid: 17525184
references.citation: Ophthalmology. 1998 May;105(5):810-24
references.pmid: 9593380
references.citation: Shock. 1999 Feb;11(2):120-6
references.pmid: 10030799
references.citation: Prev Med. 1986 Jul;15(4):373-90
references.pmid: 3532087
references.citation: Am J Physiol Gastrointest Liver Physiol. 2007 Nov;293(5):G1046-53
references.pmid: 17855766
references.citation: Int Ophthalmol Clin. 1994 Winter;34(1):27-36
references.pmid: 8169071
references.citation: Exp Aging Res. 2005 Jan-Mar;31(1):55-67
references.pmid: 15842073
references.citation: J Toxicol Clin Toxicol. 2000;38(7):755-60
references.pmid: 11192462
references.citation: Biochim Biophys Acta. 1996 Jun 7;1307(2):157-61
references.pmid: 8679700
references.citation: Dig Dis. 2003;21(3):271-5
references.pmid: 14571103
references.citation: Am J Kidney Dis. 2003 Mar;41(3 Suppl 1):S116-22
references.pmid: 12612967
references.citation: Crit Care. 2005 Oct 5;9(5):431-40
references.pmid: 16277730
references.citation: J Physiol Biochem. 2001 Dec;57(4):303-12
references.pmid: 12005033
references.citation: Proc Natl Acad Sci U S A. 1998 Aug 4;95(16):9562-6
references.pmid: 9689120
references.citation: Hum Mutat. 2000;15(3):238-45
references.pmid: 10679939
references.citation: Calcif Tissue Int. 2008 Mar;82(3):221-8
references.pmid: 18265928
references.citation: Cardiology. 2006;106(4):215-23
references.pmid: 16685128
references.citation: World J Gastroenterol. 2005 Dec 7;11(45):7197-202
references.pmid: 16437672
references.citation: Pediatr Neurol. 1990 Mar-Apr;6(2):75-81
references.pmid: 2187442
references.citation: FASEB J. 1992 Dec;6(15):3379-86
references.pmid: 1464372
references.citation: J Clin Chem Clin Biochem. 1990 May;28(5):351-7
references.pmid: 2199596
references.citation: Biochem Biophys Res Commun. 1999 Aug 2;261(2):484-7
references.pmid: 10425211
references.citation: Prog Cardiovasc Dis. 1997 Nov-Dec;40(3):265-86
references.pmid: 9406679
references.citation: Eur J Biochem. 2001 Mar;268(5):1422-9
references.pmid: 11231295
references.citation: Lancet. 1971 Feb 27;1(7696):428-30
references.pmid: 4100402
references.citation: Genes Immun. 2005 Dec;6(8):637-45
references.pmid: 16107869
references.citation: Clin Biochem. 1987 Feb;20(1):1-7
references.pmid: 3552320
references.citation: Pharmacol Res. 2006 Mar;53(3):278-86
references.pmid: 16436331
references.citation: J Inherit Metab Dis. 1999 Apr;22(2):123-31
references.pmid: 10234607
references.citation: Hum Mol Genet. 1999 Nov;8(12):2247-54
references.pmid: 10545605
references.citation: Hum Mutat. 2000 Jan;15(1):118
references.pmid: 10612840
references.citation: Proc Natl Acad Sci U S A. 2002 Feb 19;99(4):2356-61
references.pmid: 11854529
references.citation: J Gerontol A Biol Sci Med Sci. 2006 Jul;61(7):650-9
references.pmid: 16870625
references.citation: Shock. 1998 Nov;10(5):377-82
references.pmid: 9840655
references.citation: Invest Ophthalmol Vis Sci. 2008 Apr;49(4):1660-4
references.pmid: 18385088
references.citation: J Inherit Metab Dis. 1998 Jun;21(4):326-40
references.pmid: 9700590
references.citation: Am J Clin Nutr. 1989 Aug;50(2):301-6
references.pmid: 2756917
references.citation: Ann Nutr Metab. 2007;51(4):331-4
references.pmid: 17726310
references.citation: Biochim Biophys Acta. 2008 Jul-Aug;1777(7-8):564-78
references.pmid: 18406340
references.citation: Metabolism. 1977 Apr;26(4):351-6
references.pmid: 846404
references.citation: Med Hypotheses. 1984 Feb;13(2):139-51
references.pmid: 6325854
references.citation: Exp Gerontol. 1990;25(2):127-34
references.pmid: 2369927
references.citation: Am Heart J. 2000 Feb;139(2 Pt 3):S124-30
references.pmid: 10650326
references.citation: Expert Opin Investig Drugs. 2007 Dec;16(12):1921-31
references.pmid: 18042001
references.citation: Int J Clin Pharmacol Res. 1995;15(3):127-33
references.pmid: 8847154
references.citation: Nat Neurosci. 2006 Feb;9(2):227-33
references.pmid: 16415870
references.citation: Biochim Biophys Acta. 1998 Jul 31;1393(1):49-56
references.pmid: 9714731
references.citation: Ann Nutr Metab. 2008;53(1):29-32
references.pmid: 18772587
references.citation: Am J Emerg Med. 2004 Sep;22(5):335-404
references.pmid: 15490384
references.citation: Mol Cell Biochem. 2005 Dec;280(1-2):83-9
references.pmid: 16311908
references.citation: Lab Invest. 2003 Nov;83(11):1543-54
references.pmid: 14615409
references.citation: Am J Med Genet C Semin Med Genet. 2003 Aug 15;121C(1):38-52
references.pmid: 12888985
references.citation: Cornea. 1998 Jan;17(1):38-56
references.pmid: 9436879
references.citation: Lancet. 1985 Apr 6;1(8432):820-1
references.pmid: 2858698
references.citation: Br J Nutr. 2007 Nov;98(5):882-9
references.pmid: 17524183
references.citation: Cardiovasc Drugs Ther. 2007 Dec;21(6):445-8
references.pmid: 17955358
references.citation: Semin Dial. 2001 May-Jun;14(3):209-17
references.pmid: 11422928
references.citation: J Inherit Metab Dis. 2003;26(2-3):171-80
references.pmid: 12889658
references.citation: J Cell Mol Med. 2009 Apr;13(4):701-11
references.pmid: 18410524
references.citation: Nat Genet. 1999 Jan;21(1):91-4
references.pmid: 9916797
references.citation: Immunol Today. 1995 May;16(5):211-3
references.pmid: 7779248
references.citation: J Nutr. 1991 Apr;121(4):539-46
references.pmid: 2007906
references.citation: Proc Natl Acad Sci U S A. 1999 Sep 28;96(20):11341-5
references.pmid: 10500178
references.citation: Neurology. 2002 Feb 12;58(3):507-8
references.pmid: 11839873
references.citation: J Clin Invest. 2006 Apr;116(4):1081-91
references.pmid: 16528412
references.citation: Science. 1973 Nov 20;182(4115):929-31
references.pmid: 4745596
references.citation: Annu Rev Physiol. 2002;64:477-502
references.pmid: 11826276
references.citation: Semin Perinatol. 1999 Apr;23(2):152-61
references.pmid: 10331466
references.citation: Ann N Y Acad Sci. 2004 Nov;1033:52-66
references.pmid: 15591003
references.citation: J Cell Biochem. 2000 Sep 18;80(1):1-10
references.pmid: 11029749
references.citation: J Nutr. 1979 Jan;109(1):24-31
references.pmid: 430211
references.citation: Biochem Mol Biol Int. 1998 Dec;46(5):923-31
references.pmid: 9861446
references.citation: J Biol Chem. 2004 Jan 30;279(5):3817-27
references.pmid: 14615481
references.citation: Kidney Int Suppl. 1987 Oct;22:S132-5
references.pmid: 3480977
references.citation: Am J Ophthalmol. 2002 Feb;133(2):181-6
references.pmid: 11812420
references.citation: Curr Opin Investig Drugs. 2007 Apr;8(4):312-7
references.pmid: 17458181
references.citation: Calcif Tissue Int. 2005 Jun;76(6):458-65
references.pmid: 15906015
references.citation: Med Sci Monit. 2005 Jun;11(6):BR176-180
references.pmid: 15917712
references.citation: J Trop Pediatr. 1999 Oct;45(5):294-6
references.pmid: 10584472
references.citation: Res Dev Disabil. 2006 Nov-Dec;27(6):599-604
references.pmid: 16621446
references.citation: Gastroenterology. 1961 Nov;41:443-51
references.pmid: 14456652
references.citation: Pathol Int. 2008 Jul;58(7):436-41
references.pmid: 18577113
references.citation: Eur J Pharmacol. 2006 Sep 1;545(1):51-64
references.pmid: 16904103
references.citation: Nutr Clin Pract. 2008 Feb;23(1):16-34
references.pmid: 18203961
references.citation: Biochem J. 1987 Nov 15;248(1):129-37
references.pmid: 3325035
references.citation: J Child Neurol. 1995 Nov;10 Suppl 2:S8-24
references.pmid: 8576570
references.citation: Physiol Rev. 1998 Jan;78(1):247-306
references.pmid: 9457175
references.citation: Nephrology (Carlton). 2008 Feb;13(1):3-16
references.pmid: 18199095
references.citation: Phytomedicine. 2008 Aug;15(8):595-601
references.pmid: 18539446
references.citation: CNS Drugs. 2007;21 Suppl 1:13-23; discussion 45-6
references.pmid: 17696589
references.citation: Drugs Exp Clin Res. 1995;21(6):221-8
references.pmid: 8907697
references.citation: Biomed Pharmacother. 2006 Feb;60(2):77-81
references.pmid: 16472963
references.citation: Mutat Res. 2002 Nov 30;509(1-2):127-51
references.pmid: 12427535
references.citation: Dig Dis Sci. 2008 Sep;53(9):2330-3
references.pmid: 18270833
references.citation: Biochem Biophys Res Commun. 2006 Nov 24;350(3):704-8
references.pmid: 17011512
references.citation: Dig Dis Sci. 2006 Dec;51(12):2242-7
references.pmid: 17080254
references.citation: Am J Physiol Regul Integr Comp Physiol. 2008 Feb;294(2):R352-61
references.pmid: 18056987
references.citation: Proc Natl Acad Sci U S A. 2002 Feb 19;99(4):1870-5
references.pmid: 11854487
references.citation: Proc Natl Acad Sci U S A. 1999 Mar 2;96(5):2356-60
references.pmid: 10051646
references.citation: Arch Neurol. 2005 Jan;62(1):37-41
references.pmid: 15642848
references.citation: Epilepsia. 1993 Mar-Apr;34(2):332-46
references.pmid: 8453944
references.citation: Expert Opin Investig Drugs. 2003 Sep;12(9):1471-88
references.pmid: 12943492
references.citation: J Am Diet Assoc. 1994 Feb;94(2):183-8,191; quiz 189-90
references.pmid: 8300996
references.citation: J Neurol Sci. 1977 Nov;34(2):279-86
references.pmid: 925714
references.citation: Nephron Clin Pract. 2005;101(4):c174-9
references.pmid: 16103722
references.citation: Ann N Y Acad Sci. 2004 Nov;1033:132-8
references.pmid: 15591010
references.citation: Clin Chim Acta. 1977 Feb 15;75(1):163-6
references.pmid: 403033
references.citation: Am J Hypertens. 2008 Nov;21(11):1231-7
references.pmid: 18787523
references.citation: Biochem Biophys Res Commun. 1989 Jun 30;161(3):1135-43
references.pmid: 2742580
references.citation: Mayo Clin Proc. 1983 Aug;58(8):533-40
references.pmid: 6348429
references.citation: Ann Neurol. 2004 Jan;55(1):7-12
references.pmid: 14705106
references.citation: Invest Ophthalmol Vis Sci. 2008 Nov;49(11):4844-9
references.pmid: 18641280
references.citation: Am J Clin Nutr. 1984 May;39(5):738-44
references.pmid: 6424429
references.citation: Metabolism. 1992 Feb;41(2):165-71
references.pmid: 1736038
references.citation: J Ren Nutr. 2003 Jan;13(1):2-14
references.pmid: 12563618
references.citation: Blood Purif. 2006;24(1):140-2
references.pmid: 16361854
references.citation: Ann N Y Acad Sci. 2004 Nov;1033:79-91
references.pmid: 15591005
references.citation: Ann N Y Acad Sci. 2004 Nov;1033:42-51
references.pmid: 15591002
references.citation: Nat Med. 2003 Jun;9(6):756-61
references.pmid: 12754501
references.citation: Nutrition. 1997 Sep;13(9 Suppl):52S-57S
references.pmid: 9290110
references.citation: J Nutr. 1967 Mar;91(3):331-6
references.pmid: 6021815
references.citation: J Nutr. 1986 Nov;116(11):2263-9
references.pmid: 3794832
references.citation: J Biol Chem. 1999 Nov 19;274(47):33388-92
references.pmid: 10559218