The effect of carnitine on ketogenesis in perfused livers from juvenile visceral steatosis mice with systemic carnitine deficiency

Pediatric Research

Volumn 42, Issue 1, 1997, Pages 108-113

  Nakajima, Toshihiro ^a   Horiuchi, Masahisa ^b   Yamanaka, Hirofumi ^a   Kizaki, Zenro ^a   Inoue, Fumio ^a   Kodo, Naoki ^a   Kinugasa, Akihiko ^a   Saheki, Takeyori ^b   Sawada, Tadashi ^a  

^a KYOTO PREFECTURAL UNIVERSITY OF MEDICINE   (Japan)

^b KAGOSHIMA UNIVERSITY   (Japan)

Author keywords

[No Author keywords available]

Indexed keywords

BUTYRIC ACID; CARNITINE; OCTANOIC ACID; OLEIC ACID;

ANIMAL MODEL; ARTICLE; AUTOSOMAL RECESSIVE DISORDER; CARNITINE DEFICIENCY; CONTROLLED STUDY; DOSE RESPONSE; FATTY ACID OXIDATION; FATTY LIVER; INBORN ERROR OF METABOLISM; INTRAPERITONEAL DRUG ADMINISTRATION; KETOGENESIS; LIVER MITOCHONDRION; LIVER PERFUSION; MOUSE; NEWBORN; NONHUMAN; PRIORITY JOURNAL;

EID: 0030911650     PISSN: 00313998     EISSN: None     Source Type: Journal    
DOI: 10.1203/00006450-199707000-00017     Document Type: Article

References (27)

1. Koizumi T, Nikaido H, Huyakawa J, Nonomura A, Yoneda T 1988 Infantile disease with microvesicular fatty infiltration of viscera spontaneously occurring in the C3H-H-2° strain of mouse with similarities to Reye's syndrome. Lab Anim 22:83-87
2. Hayakawa J, Koizumi T, Nikaido H 1988 Inheritance of juvenile visceral steatosis (JVS) found in C3H-H-2° mice. Mouse Genome 86:261
3. Horiuchi M, Yoshida H, Kobayashi K, Kuriwaki K, Yoshimine K, Tomomura M, Koizumi T, Nikaido H, Hayakawa J, Kuwajima M, Saheki T 1993 Cardiac hypertrophy in juvenile visceral steatosis (JVS) mice with systemic carnitine deficiency. FEBS Lett 326:267-271
4. Kuwajima M, Kono N, Horiuchi M, Imamura Y, Ono A, Inui Y, Kawata S, Koizumi T, Hayakawa J, Saheki T, Tarui S 1991 Animal model of systemic carnitine deficiency: analysis in C3H-H-2° strain of mouse associated with juvenile visceral steatosis. Biochem Biophys Res Commun 174:1090-1094
5. Horiuchi M, Kobayashi K, Yamaguchi S, Shimizu N, Koizumi T, Nikaido H, Hayakawa J, Kuwajima M, Saheki T 1994 Primary defect of juvenile visceral steatosis (JVS) mouse with systemic carnitine deficiency is probably in renal carnitine transport system. Biochim Biophys Acta 1226:25-30
6. Horiuchi M, Kobayashi K, Tomomura M, Kuwajima M, Imamura Y, Koizumi T, Nikaido H, Hayakawa J, Saheki T 1992 Carnitine administration to juvenile visceral steatosis mice corrects the suppressed expression of urea cycle enzymes by normalizing their transcription. J Biol Chem 267:5032-5035
7. Bremer J 1963 Carnitine in intermediary metabolism. J Biol Chem 238:2774-2779
8. Scholte HR, Luyt-Houwen IEM, Vaandrager-Verduin MHM 1987 The role of the carnitine system in myocardial fatty acid oxidation: carnitine deficiency, failing mitochondria and cardiomyopathy. Basic Res Cardiol 82(suppl):63-73
9. Bieber LL 1988 Carnitine. Ann Rev Biochem 57:261-283
10. Bieber LL, Emaus R, Valkner K, Farrell S 1982 Possible functions of short-chain and medium-chain carnitine acyltransferases. Fed Proc 41:2858-2862
11. Scholte HR, Pereira RR, de Jonge PC, Luyt-Houwen IEM, Verduin MHM, Ross JD 1990 Primary carnitine deficiency. J Clin Chem Clin Biochem 28:351-357
12. Coulter DL 1991 Carnitine, valproate, and toxicity. J Child Neurol 6:7-14
13. Melegh B, Kemer J, Bieber LL 1987 Pivampicillin-promoted excretion of pivaloylcarnitine in humans. Biochem Pharmacol 20:3405-3409
14. Schmide-Sommerfeld E, Penn D, Wolf H 1983 Carnitine deficiency in premature infants receiving total parenteral nutrition: effect of L-carnitine supplementation. J Pediatr 102:931-935
15. Shenai JP, Borum PR, Nohan P, Donlevy SC 1983 Carnitine status at birth of newborn infants of varying gestation. Pediatr Res 17:134-137
16. Freeman JM, Vining EPG, Cost S, Singhi P 1994 Does carnitine administration improve the symptoms attributed to aniticonvulsant medications? A double-blind, crossover study. Pediatrics 93:893-895
17. Yamanaka H, Ueshima Y, Nakajima T, Yoshida N, Inoue F, Kodo N, Kinugasa A, Sawada T 1992 Gluconeogenesis and ketogenesis in perfused livers from short-chain acyl-CoA dehydrogenase-deficient mice. J Inherit Metab Dis 15:353-355
18. Assimacopoulos-Jeannet F, Exton JH, Jeanrenaud B 1973 Control of gluconeogenesis and glycogenolysis in perfused livers of normal mice. Am J Physiol 225:173-181
19. Mallette LE, Exton JH, Park CR 1969 Control of gluconeogenesis from amino acids in the perfused rat liver. J Biol Chem 244:5713-5723
20. Takahashi M, Ueda S, Misaki H, Sugiyama N, Matsumoto K, Matsuo N, Murao S 1994 Carnitine determination by an enzymatic cycling method with carnitine dehydrogenase. Clin Chem 40:817-821
21. Sandor A, Kispal G, Melegh B, Alkonyi I 1985 Release of carnitine from the perfused rat liver. Biochim Biophys Acta 835:83-91
22. Bremer J 1983 Carnitine-metabolism and function. Physiol Rev 63:1240-1280
23. Imamura Y, Saheki T, Arakawa H, Noda T, Koizumi T, Nikaido H, Hayakawa J 1990 Urea cycle disorder in C3H-H-2° mice with juvenile steatosis of viscera. FEBS Lett 260:119-121
24. McGarry JD, Leatherman GF, Foster DW 1978 Carnitine palmitoytransferase I. J Biol Chem 253:4128-4136
25. McGarry JD, Foster DW 1980 Regulation of hepatic fatty acid oxidation and ketone body production. Ann Rev Biochem 49:395-420
26. Brady LJ, Hoppel CL, Brady PS 1986 Hepatic mitochondrial inner-membrane properties, β-oxidation and carnitine palmitoyltransferases A and B. Biochem J 233:427-433
27. McGarry JD, Mills SE, Long CS, Foster DW 1983 Observations on the affinity for carnitine, and malonyl-CoA sensitivity, of carnitine palmitoyltransferase I in animal and human tissues. Biochem J 214:21-28