Slc22a5

Summary

Gene Symbol: Slc22a5
Description: solute carrier family 22 (organic cation transporter), member 5
Alias: Lstpl, Octn2, jvs, solute carrier family 22 member 5, high-affinity sodium-dependent carnitine cotransporter, juvenile visceral steatosis, organic cation/carnitine transporter 2
Species: mouse
Products:     Slc22a5

Top Publications

  1. Lahjouji K, Elimrani I, Wu J, Mitchell G, Qureshi I. A heterozygote phenotype is present in the jvs +/- mutant mouse livers. Mol Genet Metab. 2002;76:76-80 pubmed
    The juvenile visceral steatosis (jvs) mouse, having a mutation in the carnitine transporter gene Octn2, is a model of primary systemic carnitine deficiency in humans (SCD, OMIM 212140)...
  2. Nezu J, Tamai I, Oku A, Ohashi R, Yabuuchi H, Hashimoto N, et al. Primary systemic carnitine deficiency is caused by mutations in a gene encoding sodium ion-dependent carnitine transporter. Nat Genet. 1999;21:91-4 pubmed
    ..defect of the carnitine transport system in the plasma membrane in SCD patients and in the mouse model, juvenile visceral steatosis. Although the responsible loci have been mapped in both human and mouse, the underlying gene has not ..
  3. Shekhawat P, Srinivas S, Matern D, Bennett M, Boriack R, George V, et al. Spontaneous development of intestinal and colonic atrophy and inflammation in the carnitine-deficient jvs (OCTN2(-/-)) mice. Mol Genet Metab. 2007;92:315-24 pubmed
    ..b>juvenile visceral steatosis (OCTN2(-/-)) mouse spontaneously develops intestinal villous atrophy, breakdown and inflammation with ..
  4. Hirai T, Fukui Y, Motojima K. PPARalpha agonists positively and negatively regulate the expression of several nutrient/drug transporters in mouse small intestine. Biol Pharm Bull. 2007;30:2185-90 pubmed
    ..Expression levels of seven nutrient/drug transporters (Abcd3, Octn2/Slc22a5, FATP2/Slc27a2, Slc22a21, Mct13/Slc16a13, Slc23a1 and Bcrp/Abcg2) in the intestine were up-regulated and the ..
  5. Kai S, Yakushiji K, Yamauchi M, Ito C, Kuwajima M, Osada Y, et al. Expression of novel organic cation/carnitine transporter (OCTN2) in the mouse pancreas. Tissue Cell. 2005;37:309-15 pubmed
    Among the organic cation transporters, OCTN2 is identified as the most important carnitine transporter owing to the ability to transport carnitine...
  6. Ushikai M, Horiuchi M, Kobayashi K, Matuda S, Inui A, Takeuchi T, et al. Induction of PDK4 in the heart muscle of JVS mice, an animal model of systemic carnitine deficiency, does not appear to reduce glucose utilization by the heart. Mol Genet Metab. 2011;102:349-55 pubmed publisher
    ..mRNA has been reported as an up-regulated gene in the heart and skeletal muscle of carnitine-deficient juvenile visceral steatosis (JVS) mice under fed conditions...
  7. Tomomura M, Nakagawa K, Saheki T. Proto-oncogene c-jun and c-fos messenger RNAs increase in the liver of carnitine-deficient juvenile visceral steatosis (jvs) mice. FEBS Lett. 1992;311:63-6 pubmed
    We determined the mRNA levels of c-jun and c-fos in the liver of C3H-H-2 degrees jvs mice. Both were higher in jvs mice than in normal mice...
  8. Cossette S, Gastonguay A, Bao X, Lerch Gaggl A, Zhong L, Harmann L, et al. Sucrose non-fermenting related kinase enzyme is essential for cardiac metabolism. Biol Open. 2014;4:48-61 pubmed publisher
    ..Our results suggest that Snrk is essential for maintaining cardiac metabolic homeostasis, and shows an autonomous role for SNRK during mammalian development. ..
  9. Tamai I, Ohashi R, Nezu J, Sai Y, Kobayashi D, Oku A, et al. Molecular and functional characterization of organic cation/carnitine transporter family in mice. J Biol Chem. 2000;275:40064-72 pubmed
    ..We have already shown that a defect of the organic cation/carnitine transporter OCTN2 is a primary cause of systemic carnitine deficiency...

More Information

Publications66

  1. Inano A, Sai Y, Nikaido H, Hasimoto N, Asano M, Tsuji A, et al. Acetyl-L-carnitine permeability across the blood-brain barrier and involvement of carnitine transporter OCTN2. Biopharm Drug Dispos. 2003;24:357-65 pubmed
    OCTN2 (SLC22A5), an organic cation/carnitine transporter, is widely distributed throughout the body, including the brain...
  2. Suenaga M, Kuwajima M, Himeda T, Morokami K, Matsuura T, Ozaki K, et al. Identification of the up- and down-regulated genes in the heart of juvenile visceral steatosis mice. Biol Pharm Bull. 2004;27:496-503 pubmed
    b>Juvenile visceral steatosis (JVS) mice, novel animal models of systemic carnitine deficiency, exhibit a remarkably increased number of mitochondria in their cardiac myocytes...
  3. Iwata D, Kato Y, Wakayama T, Sai Y, Kubo Y, Iseki S, et al. Involvement of carnitine/organic cation transporter OCTN2 (SLC22A5) in distribution of its substrate carnitine to the heart. Drug Metab Pharmacokinet. 2008;23:207-15 pubmed
    ..L-[3H]Carnitine uptake by heart slices from juvenile visceral steatosis (jvs) mice, which have a hereditary octn2 gene deficiency, was negligible...
  4. Lamhonwah A, Wong J, Tam C, Mai L, Tein I. Organic cation/carnitine transporter family expression patterns in adult murine heart. Pathol Res Pract. 2009;205:395-402 pubmed publisher
    ..This distribution may play a role in the hypertrophic cardiomyopathy seen in hOCTN2 deficiency, and may also affect the absorption/elimination of organic cationic cardiac drugs. ..
  5. Sakoguchi T, Horiuchi M, Asakawa A, Ushikai M, Yoshida G, Fujimiya M, et al. Failure of the feeding response to fasting in carnitine-deficient juvenile visceral steatosis (JVS) mice: involvement of defective acyl-ghrelin secretion and enhanced corticotropin-releasing factor signaling in the hypothalamus. Biochim Biophys Acta. 2009;1792:1087-93 pubmed publisher
    Carnitine-deficient juvenile visceral steatosis (JVS) mice, suffering from fatty acid metabolism abnormalities, have reduced locomotor activity after fasting...
  6. Horiuchi M, Kobayashi K, Yamaguchi S, Shimizu N, Koizumi T, Nikaido H, et al. Primary defect of juvenile visceral steatosis (jvs) mouse with systemic carnitine deficiency is probably in renal carnitine transport system. Biochim Biophys Acta. 1994;1226:25-30 pubmed
    ..reabsorptional system for carnitine in the kidney to elucidate the mechanism of carnitine deficiency in juvenile visceral steatosis (jvs) mice...
  7. Li M, Yoshida G, Horiuchi M, Kobayashi K, Saheki T. Prolonged effect of single carnitine administration on fasted carnitine-deficient JVS mice regarding their locomotor activity and energy expenditure. Biochim Biophys Acta. 2006;1761:1191-9 pubmed
    ..of carnitine on the reduced locomotor activity and energy metabolism of fasted carnitine-deficient juvenile visceral steatosis (jvs(-/-)) mice...
  8. Wen G, Ringseis R, Eder K. Mouse OCTN2 is directly regulated by peroxisome proliferator-activated receptor alpha (PPARalpha) via a PPRE located in the first intron. Biochem Pharmacol. 2010;79:768-76 pubmed publisher
    Recent studies provided strong evidence to suggest that organic cation transporter 2 (OCTN2) is a direct target gene of peroxisome proliferator-activated receptor alpha (PPARalpha)...
  9. Cotton L, Rodriguez C, Suzuki K, Orgebin Crist M, Hinton B. Organic cation/carnitine transporter, OCTN2, transcriptional activity is regulated by osmotic stress in epididymal cells. Mol Reprod Dev. 2010;77:114-25 pubmed publisher
    ..Further, it was also seen that OCTN2, a transporter that is thought to be responsible for the accumulation of L-carnitine in the epididymal lumen, is ..
  10. Toshimori K, Kuwajima M, Yoshinaga K, Wakayama T, Shima K. Dysfunctions of the epididymis as a result of primary carnitine deficiency in juvenile visceral steatosis mice. FEBS Lett. 1999;446:323-6 pubmed
    The juvenile visceral steatosis mutant mice serve as an animal model of primary carnitine deficiency, classified as the sudden infant death syndrome...
  11. Takahashi R, Asai T, Murakami H, Murakami R, Tsuzuki M, Numaguchi Y, et al. Pressure overload-induced cardiomyopathy in heterozygous carrier mice of carnitine transporter gene mutation. Hypertension. 2007;50:497-502 pubmed
    ..induced pressure overload on the hearts of heterozygous mutants of a murine model of OCTN2 mutation, juvenile visceral steatosis mouse (jvs/+). Eleven-week-old jvs/+ mice and age-matched wild-type mice were used...
  12. Cui J, Gunewardena S, Yoo B, Liu J, Renaud H, Lu H, et al. RNA-Seq reveals different mRNA abundance of transporters and their alternative transcript isoforms during liver development. Toxicol Sci. 2012;127:592-608 pubmed publisher
    ..In conclusion, this study reveals the mRNA abundance of transporters in liver and demonstrates that the expression of liver transporters is both age and isoform specific. ..
  13. Shekhawat P, Yang H, Bennett M, Carter A, Matern D, Tamai I, et al. Carnitine content and expression of mitochondrial beta-oxidation enzymes in placentas of wild-type (OCTN2(+/+)) and OCTN2 Null (OCTN2(-/-)) Mice. Pediatr Res. 2004;56:323-8 pubmed
    ..the role of carnitine in the expression of the enzymes involved in fatty acid beta-oxidation in placenta of OCTN2(-/-) mice with defective carnitine transporter (OCTN2)...
  14. Okita K, Tokino T, Nishimori H, Miura K, Nikaido H, Hayakawa J, et al. Definition of the locus responsible for systemic carnitine deficiency within a 1.6-cM region of mouse chromosome 11 by detailed linkage analysis. Genomics. 1996;33:289-91 pubmed
    ..The juvenile visceral steatosis (JVS) mouse, an animal model of systematic carnitine deficiency, inherits the JVS phenotype in ..
  15. Hashimoto N, Suzuki F, Tamai I, Nikaido H, Kuwajima M, Hayakawa J, et al. Gene-dose effect on carnitine transport activity in embryonic fibroblasts of JVS mice as a model of human carnitine transporter deficiency. Biochem Pharmacol. 1998;55:1729-32 pubmed
    ..in renal carnitine reabsorption has been thought to account fotr the systemic carnitine deficiency in juvenile visceral steatosis (JVS) mice...
  16. Horiuchi M, Yoshida H, Kobayashi K, Kuriwaki K, Yoshimine K, Tomomura M, et al. Cardiac hypertrophy in juvenile visceral steatosis (jvs) mice with systemic carnitine deficiency. FEBS Lett. 1993;326:267-71 pubmed
    We have reported the clinical and biochemical findings in juvenile visceral steatosis (jvs) mice with systemic carnitine deficiency. This paper is the first report about cardiomyopathy in jvs mice...
  17. D Argenio G, Petillo O, Margarucci S, Torpedine A, Calarco A, Koverech A, et al. Colon OCTN2 gene expression is up-regulated by peroxisome proliferator-activated receptor gamma in humans and mice and contributes to local and systemic carnitine homeostasis. J Biol Chem. 2010;285:27078-87 pubmed publisher
    In the large intestine organic cation transporter type-2 (OCTN2) is recognized as a transporter of compounds such as carnitine and colony sporulation factor, promoting health of the colon intestinal epithelium...
  18. Suenaga M, Arakaki N, Morokami K, Himeda T, Shibata H, Kuwajima M, et al. Functional disorders of the oxidative phosphorylation system in the heart mitochondria of mice with juvenile visceral steatosis. Biol Pharm Bull. 2003;26:289-94 pubmed
    Mice with juvenile visceral steatosis (JVS) develop remarkable cardiac hypertrophy and exhibit an increased number of mitochondria in their heart...
  19. Peng J, Yu L, Horiuchi M, Zhang P, Huang X, Zhang Y, et al. Identification of human CDV-1R and mouse Cdv-1R, two novel proteins with putative signal peptides, especially highly expressed in testis and increased with the male sex maturation. Mol Biol Rep. 2002;29:353-62 pubmed
    Human systemic carnitine deficiency (SCD) is a hereditary disease caused by the mutation of OCTN2 and has the characteristics of cardiac hypertrophy...
  20. Lamhonwah A, Hawkins C, Tam C, Wong J, Mai L, Tein I. Expression patterns of the organic cation/carnitine transporter family in adult murine brain. Brain Dev. 2008;30:31-42 pubmed
  21. Kato Y, Sai Y, Yoshida K, Watanabe C, Hirata T, Tsuji A. PDZK1 directly regulates the function of organic cation/carnitine transporter OCTN2. Mol Pharmacol. 2005;67:734-43 pubmed
    ..C-terminal proteins of OCTs and OCTNs identified a specific interaction of apical transporters OCTN1 and OCTN2, but not basolateral transporters OCT1 and OCT2, with PDZK1, intestinal and kidney-enriched PDZ protein, and Na+/H+..
  22. van Vlies N, Ferdinandusse S, Turkenburg M, Wanders R, Vaz F. PPAR alpha-activation results in enhanced carnitine biosynthesis and OCTN2-mediated hepatic carnitine accumulation. Biochim Biophys Acta. 2007;1767:1134-42 pubmed
    ..carnitine biosynthesis enzyme activities, levels of carnitine biosynthesis intermediates, acyl-carnitines and OCTN2 mRNA levels in tissues of untreated, fasted or Wy-14643-treated wild type and PPAR alpha-/- mice...
  23. Toledo F, Liu C, Lee C, Wahl G. RMCE-ASAP: a gene targeting method for ES and somatic cells to accelerate phenotype analyses. Nucleic Acids Res. 2006;34:e92 pubmed
    ..These general principles should make RMCE-ASAP applicable to any locus. ..
  24. Sugiura T, Kato Y, Wakayama T, Silver D, Kubo Y, Iseki S, et al. PDZK1 regulates two intestinal solute carriers (Slc15a1 and Slc22a5) in mice. Drug Metab Dispos. 2008;36:1181-8 pubmed publisher
    ..protein, PDZK1, as a regulatory mechanism of two solute carriers, Slc15a1 (oligopeptide transporter PEPT1) and Slc22a5 (carnitine/organic cation transporter OCTN2) in mouse small intestine by using pdzk1 gene knockout (pdzk1(-/-)) ..
  25. Monte J, Nagle M, Eraly S, Nigam S. Identification of a novel murine organic anion transporter family member, OAT6, expressed in olfactory mucosa. Biochem Biophys Res Commun. 2004;323:429-36 pubmed
    ..Furthermore, e-blot data suggest very different expression of individual OATs, OCTs and OCTNs in kidney, brain, liver, and eye. ..
  26. Wu X, Huang W, Prasad P, Seth P, Rajan D, Leibach F, et al. Functional characteristics and tissue distribution pattern of organic cation transporter 2 (OCTN2), an organic cation/carnitine transporter. J Pharmacol Exp Ther. 1999;290:1482-92 pubmed
    ..b>OCTN2 transports organic cations without involving Na(+), but it transports carnitine only in the presence of Na(+)...
  27. Nakajima T, Horiuchi M, Yamanaka H, Kizaki Z, Inoue F, Kodo N, et al. The effect of carnitine on ketogenesis in perfused livers from juvenile visceral steatosis mice with systemic carnitine deficiency. Pediatr Res. 1997;42:108-13 pubmed
    b>Juvenile visceral steatosis (JVS) mice have been reported to have systemic carnitine deficiency, and the carnitine concentration in the liver of JVS mice was markedly lower than that of controls (11.6 +/- 2.6 versus 393.5 +/- 56...
  28. Sonne S, Shekhawat P, Matern D, Ganapathy V, Ignatowicz L. Carnitine deficiency in OCTN2-/- newborn mice leads to a severe gut and immune phenotype with widespread atrophy, apoptosis and a pro-inflammatory response. PLoS ONE. 2012;7:e47729 pubmed publisher
    ..of carnitine deficiency in the neonatal gut using a mouse model with a loss-of-function mutation in the OCTN2 (SLC22A5) carnitine transporter...
  29. Tomomura M, Tomomura A, Musa D, Horiuchi M, Takiguchi M, Mori M, et al. Suppressed expression of the urea cycle enzyme genes in the liver of carnitine-deficient juvenile visceral steatosis (JVS) mice in infancy and during starvation in adulthood. J Biochem. 1997;121:172-7 pubmed
    Systemic carnitine-deficient juvenile visceral steatosis (JVS) mice exhibit decreased expression of some liver-selective genes including those for the urea cycle enzymes during the infantile period...
  30. Uenaka R, Kuwajima M, Ono A, Matsuzawa Y, Hayakawa J, Inohara N, et al. Increased expression of carnitine palmitoyltransferase I gene is repressed by administering L-carnitine in the hearts of carnitine-deficient juvenile visceral steatosis mice. J Biochem. 1996;119:533-40 pubmed
    The juvenile visceral steatosis (JVS) mouse is a novel mutant animal for studying systemic carnitine deficiency...
  31. Koizumi T, Nikaido H, Hayakawa J, Nonomura A, Yoneda T. Infantile disease with microvesicular fatty infiltration of viscera spontaneously occurring in the C3H-H-2(0) strain of mouse with similarities to Reye's syndrome. Lab Anim. 1988;22:83-7 pubmed
    ..The clinical, biochemical and histopathological findings of an infantile disease occurring in the C3H-H-2 degree strain of mice, which has similarities with Reye's syndrome in children, is described. ..
  32. Horiuchi M, Kobayashi K, Masuda M, Terazono H, Saheki T. Pyruvate dehydrogenase kinase 4 mRNA is increased in the hypertrophied ventricles of carnitine-deficient juvenile visceral steatosis (JVS) mice. Biofactors. 1999;10:301-9 pubmed
    ..4 (PDK4) with differential mRNA display as an up-regulated gene in the hypertrophied ventricles of juvenile visceral steatosis (JVS) mice with systemic carnitine deficiency...
  33. Tomomura M, Tomomura A, Dewan M, Saheki T. Long-chain fatty acids suppress the induction of urea cycle enzyme genes by glucocorticoid action. FEBS Lett. 1996;399:310-2 pubmed
    ..acids are the mediator of the abnormal expression of urea cycle enzyme genes in carnitine-deficient juvenile visceral steatosis (JVS) mice, the effects of fatty acids on urea cycle enzyme, carbamoylphosphate synthetase (CPS) and ..
  34. Tomomura M, Imamura Y, Tomomura A, Horiuchi M, Saheki T. Abnormal gene expression and regulation in the liver of jvs mice with systemic carnitine deficiency. Biochim Biophys Acta. 1994;1226:307-14 pubmed
    Carnitine-deficient jvs mice expressed reduced levels of a group of genes which are preferentially expressed in the liver, including urea cycle enzyme genes (Biochim. Biophys. Acta 1138, 167-171, 1992)...
  35. Miyagawa J, Kuwajima M, Hanafusa T, Ozaki K, Fujimura H, Ono A, et al. Mitochondrial abnormalities of muscle tissue in mice with juvenile visceral steatosis associated with systemic carnitine deficiency. Virchows Arch. 1995;426:271-9 pubmed
    A mouse with juvenile visceral steatosis (the JVS mouse) has been recognized as a novel animal model for systemic carnitine deficiency...
  36. Nikaido H, Horiuchi M, Hashimoto N, Saheki T, Hayakawa J. Mapping of jvs (juvenile visceral steatosis) gene, which causes systemic carnitine deficiency in mice, on chromosome 11. Mamm Genome. 1995;6:369-70 pubmed
  37. Hotta K, Kuwajima M, Ono A, Nakajima H, Horikawa Y, Miyagawa J, et al. Disordered expression of glycolytic and gluconeogenic liver enzymes of juvenile visceral steatosis mice with systemic carnitine deficiency. Diabetes Res Clin Pract. 1996;32:117-23 pubmed
    ..of carnitine deficiency on expression of glycolytic and gluconeogenic enzymes was performed using juvenile visceral steatosis mice which are systemically deficient in carnitine...
  38. Hotta K, Kuwajima M, Ono A, Uenaka R, Nakajima H, Miyagawa J, et al. Altered expression of carnitine palmitoyltransferase II in liver, muscle, and heart of mouse strain with juvenile visceral steatosis. Biochim Biophys Acta. 1996;1289:131-5 pubmed
    ..on the mRNA level of carnitine palmitoyltransferase II in the liver, muscle and heart of mice with juvenile visceral steatosis, a strain that is systematically deficient in carnitine...
  39. Kuwajima M, Lu K, Harashima H, Ono A, Sato I, Mizuno A, et al. Carnitine transport defect in fibroblasts of juvenile visceral steatosis (JVS) mouse. Biochem Biophys Res Commun. 1996;223:283-7 pubmed
    b>Juvenile visceral steatosis (JVS) mice are associated with systemic carnitine deficiency (Kuwajima, et al., 1991)...
  40. Grube M, Ameling S, Noutsias M, Köck K, Triebel I, Bonitz K, et al. Selective regulation of cardiac organic cation transporter novel type 2 (OCTN2) in dilated cardiomyopathy. Am J Pathol. 2011;178:2547-59 pubmed publisher
    ..Genetic variants of OCTN2, for example, reduce uptake of carnitine, leading to heart failure...
  41. Tomomura M, Imamura Y, Horiuchi M, Koizumi T, Nikaido H, Hayakawa J, et al. Abnormal expression of urea cycle enzyme genes in juvenile visceral steatosis (jvs) mice. Biochim Biophys Acta. 1992;1138:167-71 pubmed
    b>Juvenile visceral steatosis (jvs) mice from the C3H-H-2 degrees strain have markedly low levels of all the hepatic urea cycle enzymes (Imamura et al. (1990) FEBS Lett. 260, 119-121)...
  42. Masuda M, Kobayashi K, Horiuchi M, Terazono H, Yoshimura N, Saheki T. A novel gene suppressed in the ventricle of carnitine-deficient juvenile visceral steatosis mice. FEBS Lett. 1997;408:221-4 pubmed
    In order to clarify the pathogenesis and pathophysiology of cardiac hypertrophy in carnitine-deficient juvenile visceral steatosis (JVS) mice, we performed mRNA differential display analysis with total RNA extracted from the ventricles ..
  43. Koch A, König B, Stangl G, Eder K. PPAR alpha mediates transcriptional upregulation of novel organic cation transporters-2 and -3 and enzymes involved in hepatic carnitine synthesis. Exp Biol Med (Maywood). 2008;233:356-65 pubmed publisher
    ..Wild-type mice treated with WY 14,643 had a greater abundance of OCTN2 mRNA in their liver, muscle, kidney, and small intestine and a greater abundance of OCTN3 mRNA in kidney and small ..
  44. Lamhonwah A, Skaug J, Scherer S, Tein I. A third human carnitine/organic cation transporter (OCTN3) as a candidate for the 5q31 Crohn's disease locus (IBD5). Biochem Biophys Res Commun. 2003;301:98-101 pubmed
    ..The murine organic cation/carnitine (Octn) transporter family, Octn1, Octn2, and Octn3 is clustered on mouse chromosome 11 (NCBI Accession No. NW_000039)...
  45. Ramsay R, Gandour R, van der Leij F. Molecular enzymology of carnitine transfer and transport. Biochim Biophys Acta. 2001;1546:21-43 pubmed
    ..It is found in most cells at millimolar levels after uptake via the sodium-dependent carrier, OCTN2. The acylation state of the mobile carnitine pool is linked to that of the limited and compartmentalised coenzyme ..
  46. Zhu Y, Jong M, Frazer K, Gong E, Krauss R, Cheng J, et al. Genomic interval engineering of mice identifies a novel modulator of triglyceride production. Proc Natl Acad Sci U S A. 2000;97:1137-42 pubmed
    ..These studies revealed that OCTN2, a gene recently shown to play a role in carnitine transport, was able to correct the triglyceride abnormalities...
  47. Abdullah Abu Musa D, Kobayashi K, Yasuda I, Iijima M, Christoffels V, Tomomura M, et al. Involvement of a cis-acting element in the suppression of carbamoyl phosphate synthetase I gene expression in the liver of carnitine-deficient mice. Mol Genet Metab. 1999;68:346-56 pubmed
    ..of carbamoyl phosphate synthetase I (CPS) gene is suppressed in the liver of carnitine-deficient juvenile visceral steatosis (JVS) mice at weaning and under starvation at adult age...
  48. Yokogawa K, Higashi Y, Tamai I, Nomura M, Hashimoto N, Nikaido H, et al. Decreased tissue distribution of L-carnitine in juvenile visceral steatosis mice. J Pharmacol Exp Ther. 1999;289:224-30 pubmed
    We kinetically analyzed the disposition of L-carnitine of juvenile visceral steatosis (JVS) mice compared with that of normal mice to elucidate the mechanism of the systemic L-carnitine deficiency of JVS mice...
  49. Lu K, Nishimori H, Nakamura Y, Shima K, Kuwajima M. A missense mutation of mouse OCTN2, a sodium-dependent carnitine cotransporter, in the juvenile visceral steatosis mouse. Biochem Biophys Res Commun. 1998;252:590-4 pubmed
    Carnitine is an essential cofactor for the mitochondrial beta-oxidation of long-chain fatty acids. The juvenile visceral steatosis (JVS) mouse, an animal model of systemic carnitine deficiency, is inherited in an autosomal recessive ..
  50. Frazer K, Ueda Y, Zhu Y, Gifford V, Garofalo M, Mohandas N, et al. Computational and biological analysis of 680 kb of DNA sequence from the human 5q31 cytokine gene cluster region. Genome Res. 1997;7:495-512 pubmed
  51. Yoshimine K, Horiuchi M, Suzuki S, Kobayashi K, Abdul J, Masuda M, et al. Altered expression of atrial natriuretic peptide and contractile protein genes in hypertrophied ventricle of JVS mice with systemic carnitine deficiency. J Mol Cell Cardiol. 1997;29:571-8 pubmed
    To characterize cardiac hypertrophy in juvenile visceral steatosis (JVS) mice with systemic carnitine deficiency, we investigated how the hypertrophy develops and whether it is associated with altered expression of any specific genes, ..
  52. Kuwajima M, Kono N, Horiuchi M, Imamura Y, Ono A, Inui Y, et al. Animal model of systemic carnitine deficiency: analysis in C3H-H-2 degrees strain of mouse associated with juvenile visceral steatosis. Biochem Biophys Res Commun. 1991;174:1090-4 pubmed
    ..This is a useful animal model to analyze the role of carnitine in lipid, amino acid and carbohydrate metabolism. ..
  53. Kato Y, Sugiura M, Sugiura T, Wakayama T, Kubo Y, Kobayashi D, et al. Organic cation/carnitine transporter OCTN2 (Slc22a5) is responsible for carnitine transport across apical membranes of small intestinal epithelial cells in mouse. Mol Pharmacol. 2006;70:829-37 pubmed
    ..for a predominant role of OCTN2 in small intestinal absorption of carnitine based on experiments with juvenile visceral steatosis (jvs) mice, which have a hereditary deficiency of the octn2 gene...
  54. Yakushiji K, Kai S, Yamauchi M, Kuwajima M, Osada Y, Toshimori K. Expression and distribution of OCTN2 in mouse epididymis and its association with obstructive azoospermia in juvenile visceral steatosis mice. Int J Urol. 2006;13:420-6 pubmed
    ..We reported that obstructive azoospermia occurred in the epididymis in the juvenile visceral steatosis (JVS) mice, which are OCTN2 dysfunction mice caused by mutations in the gene encoding OCTN2, have been ..
  55. Yoshida G, Li M, Horiuchi M, Nakagawa S, Sakata M, Kuchiiwa S, et al. Fasting-induced reduction in locomotor activity and reduced response of orexin neurons in carnitine-deficient mice. Neurosci Res. 2006;55:78-86 pubmed
    We found reduced locomotor activity (LA) under fasting in systemic carnitine-deficient juvenile visceral steatosis (jvs(-/-)) mice. When food was withdrawn at 8:00 a.m. (lights-off at 7:00 p.m...
  56. Yokogawa K, Yonekawa M, Tamai I, Ohashi R, Tatsumi Y, Higashi Y, et al. Loss of wild-type carrier-mediated L-carnitine transport activity in hepatocytes of juvenile visceral steatosis mice. Hepatology. 1999;30:997-1001 pubmed
    b>Juvenile visceral steatosis (JVS) mice, which show systemic L-carnitine deficiency, may be an animal model of Reye's syndrome because of its phenotype of fat deposition and mitochondrial abnormalities in the liver...
  57. Ozaki K, Sano T, Tsuji N, Matsuura T, Narama I. Carnitine is necessary to maintain the phenotype and function of brown adipose tissue. Lab Invest. 2011;91:704-10 pubmed publisher
    The juvenile visceral steatosis (JVS) mouse is a mutant strain with an inherited systemic carnitine deficiency. Mice of this strain show clinical signs attributable to impaired heat production and disturbed energy production...