Liver-specific silencing of the human gene encoding succinyl-CoA: 3-Ketoacid CoA transferase

Tohoku Journal of Experimental Medicine

Volumn 215, Issue 3, 2008, Pages 227-236

  Orii, Kenji E ^a   Fukao, Toshiyuki ^a   Song, Xiang Qian ^a   Mitchell, Grant A ^b   Kondo, Naomi ^a  

^a GIFU UNIVERSITY GRADUATE SCHOOL OF MEDICINE   (Japan)

^b UNIVERSITY OF MONTREAL   (Canada)

Author keywords

3 ketoacid CoA transferase; Gene regulation; Spl; Succinyl CoA

Indexed keywords

3 OXOACID COENZYME A TRANSFERASE; ACETOACETIC ACID; LUCIFERASE; TRANSCRIPTION FACTOR SP1;

5' UNTRANSLATED REGION; ARTICLE; CANCER CELL CULTURE; CELL STRAIN HEPG2; CONTROLLED STUDY; DNA FLANKING REGION; ENERGY METABOLISM; GENE EXPRESSION; GENE FUNCTION; GENE SILENCING; HELA CELL; HUMAN; HUMAN CELL; HUMAN TISSUE; IMMUNOBLOTTING; IN VITRO STUDY; KETOGENESIS; LIVER CELL; PROMOTER REGION; TRANSCRIPTION REGULATION;

BASE SEQUENCE; CELL LINE, TUMOR; CLONING, MOLECULAR; COENZYME A-TRANSFERASES; DNA PRIMERS; GENE EXPRESSION REGULATION, ENZYMOLOGIC; GENE SILENCING; HELA CELLS; HUMANS; KETONES; LIVER; MITOCHONDRIA; MODELS, GENETIC; MOLECULAR SEQUENCE DATA; PROMOTER REGIONS (GENETICS);

EID: 48949083339     PISSN: 00408727     EISSN: 13493329     Source Type: Journal    
DOI: 10.1620/tjem.215.227     Document Type: Article

References (19)

1. Dignam, J.D., Lebovitz, R.M. & Roeder, R.G. (1983) Accurate transcription initiation by RNA polymerase II in a soluble extract from isolated mammalian nuclei. Nucleic. Acids Res., 11, 1475-1489.
2. Fenselau, A., Wallis, K. & Morris, H.P. (1976) Subcellular localization of acetoacetate coenzyme A transferase in rat hepatomas. Cancer Res., 36, 4429-4433.
3. Fields, A.L.A., Wolman, S.L., Cheema-Dhadli, S., Morris, H.P. & Halperin, M.L. (1981) Regulation of energy metabolism in Morris hepatoma 7777 and 7800. Cancer Res., 41, 2762-2766.
4. Fukao, T., Song' X.-Q., Mitchell, G.A., Yamaguchi, S., Sukegawa, K., Orii, T. & Kondo, N. (1997) Enzyme of ketone body utilization in human tissues: Protein and messenger RNA levels of succinyl-Coenzyme A (CoA): 3-ketoacid CoA transferase and mitochondrial and cytosolic acetoacetyl-CoA thiolases. Pediatr. Res., 42, 498-502.
5. Fukao, T., Kaneko, H., Birrell, G., Gatei, M., Tashita, H., Yoshida, T., Cross, S., Kedar, P., Watters, D., Khana, K.K., Misko, I., Kondo, N. & Lavin, M.F. (1999) ATM is upregulated during the mitogenic response in peripheral blood mononuclear cells. Blood, 94, 1998-2006.
6. Fukao, T., Mitchell, G.A., Song, X.Q., Nakamura, H., Kassovska-Bratinova, S., Orii, K.E., Wraith, J.E., Besley, G., Wanders, R.J.A., Niezen-Koning, K.E., Berry, G.T., Palmieri, M. & Kondo, N. (2000) Succinyl-CoA:3-ketoacid CoA transferase (SCOT): Cloning of the human SCOT gene, tertiary structural modeling of the human SCOT monomer, and characterization of three Pathogenic mutations. Genomics, 68, 144-151.
7. Kassovska-Bratinova, S., Fukao, T., Song, X.-Q., Duncan, A., Chen, H.S., Robert, M.-F., Perez-Cerda, C., Ugarte, M., Chartrand, R, Vobecky, S., Kondo, N. & Mitchell, G.A. (1996) Succinyl-CoA: 3-ketoacid CoA transferase (SCOT): Human SCOT cDNA cloning and chromosomal Tapping and mutation detection in a SCOT-deficient patient. Am. L Hum. Genet., 59, 519-528.
8. Lavin, T.N., Baxter, J.D. & Horita, S. (1988) The thyroid hormone receptor binds to multiple domains of the rat growth hormone 5′-flanking sequence. J. Biol. Chem., 263, 9418-9426.
9. Lowry, O.H., Rosebrough, N.J., Farr, A.L. & Randall, R.J. (1951) Protein measurement with the Folin phenol reagent. J. Biol. Chem., 193, 265-275.
10. Mitchell, G.A. & Fukao, T. (2001) Chapter102 Inborn errors of ketone body catabolism. In: Molecular and Metabolic Bases of Inherited Disease, 8th ed., edited by C.R. Scriver, A.L. Beaudet, W.S. Sly & D. Valle, NewYork McGraw-Hill, Inc. pp. 2327-2356.
11. Miyamoto, T., Suzuki, S. & Degroot, L.J. (1993) High affinity and specificity of dimeric binding of thyroid hormone receptors to DNA and their ligand-dependent dissociation. Mol. Endcrinol., 7, 224-231.
12. Page, M.A., Krebs, H.A. & Williamson, D.H. (1971) Activities of enzymes of ketone-body utilization in brain and other tissues of suckling rats. Biochem. J., 121, 49-53.
13. Sakazaki, H., Hirayama, K., Murakami, S., Yonezawa, S., Shintaku, H., Sawada, Y., Fukao, T, Watanabe, H., Orii, T. & Isshiki, G. (1995) A new Japanese case of succinyl-CoA: 3-ketoacid CoA-transferase deficiency. J. Inherit. Metab. Dis., 18, 323-325.
14. Sambrook, J., Fritsch, E.F. & Maniatis, T. (1989) Molecular Cloning: A Laboratory Manual, 2nd Ed., Cold Spring Harbor Laboratory, Cold Spring Harbor, NY.
15. Song, X.-Q., Fukao, T., Mitchell, G.A., Kassovska-Bratinova, S., Ugarte, M., Wanders, R.J.A., Hirayama, K., Shintaku, H., Churchill, P., Watanabe, H., Orii, T. & Kondo, N. (1997) Succinyl-CoA: 3-ketoacid coenzyme A transferase (SCOT): Development of an antibody to human SCOT and diagnostic use in hereditary SCOT deficiency. Biochim. Biophys. Acta, 1360, 151-156.
16. Song, X.-Q., Fukao, T., Watanabe, H., Shintaku, H., Hirayama, K., Kassovska-Bratinova, A., Kondo, N. & Mitchell, G.A. (1998) Succinyl-CoA: 3-ketoacid CoA transferase (SCOT) deficiency: Two pathogenic mutations, V133E and C456F, in Japanese siblings. Hum. Mut., 12, 83-88.
17. Tashita, H., Fukao, T., Kaneko, H., Teramoto, T., Inoue, R., Kasahara, K. & Kondo, N. (1998) Molecular basis of selective IgG2 deficiency: The mutated membrane-bound form of gamma2 heavy chain caused complete IgG2 deficiency in two Japanese siblings. J. Clin. Invest., 101, 677-681.
18. Williamson, D.H., Bates, M.W., Ann Page, M. & Krebs, H.A. (1971) Activities of enzymes involved in acetoacetate utilization in adult mammalian tissues. Biochem. J., 121, 41-47.
19. Zhang, W.W., Lindahl, R. & Churchill, P. (1990) Regulation of succinyl coenzyme A: Acetoacetyl coenzyme A transferase in rat hepatoma cell lines. Cancer Res., 50, 5858-5862.