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Items: 1 to 20 of 111

1.

Ketone body metabolism and its defects.

Fukao T, Mitchell G, Sass JO, Hori T, Orii K, Aoyama Y.

J Inherit Metab Dis. 2014 Jul;37(4):541-51. doi: 10.1007/s10545-014-9704-9. Review.

PMID:
24706027
2.

Inborn errors of ketone body utilization.

Hori T, Yamaguchi S, Shinkaku H, Horikawa R, Shigematsu Y, Takayanagi M, Fukao T.

Pediatr Int. 2015;57(1):41-8. doi: 10.1111/ped.12585. Review.

PMID:
25559898
3.

Successful adaptation to ketosis by mice with tissue-specific deficiency of ketone body oxidation.

Cotter DG, Schugar RC, Wentz AE, d'Avignon DA, Crawford PA.

Am J Physiol Endocrinol Metab. 2013 Feb 15;304(4):E363-74. doi: 10.1152/ajpendo.00547.2012.

4.
5.

A neonatal-onset succinyl-CoA:3-ketoacid CoA transferase (SCOT)-deficient patient with T435N and c.658-666dupAACGTGATT p.N220_I222dup mutations in the OXCT1 gene.

Fukao T, Ishii T, Amano N, Kursula P, Takayanagi M, Murase K, Sakaguchi N, Kondo N, Hasegawa T.

J Inherit Metab Dis. 2010 Dec;33 Suppl 3:S307-13. doi: 10.1007/s10545-010-9168-5.

PMID:
20652411
6.

The mitochondrial acetoacetyl-CoA thiolase (T2) deficiency in Japanese patients: urinary organic acid and blood acylcarnitine profiles under stable conditions have subtle abnormalities in T2-deficient patients with some residual T2 activity.

Fukao T, Zhang GX, Sakura N, Kubo T, Yamaga H, Hazama A, Kohno Y, Matsuo N, Kondo M, Yamaguchi S, Shigematsu Y, Kondo N.

J Inherit Metab Dis. 2003;26(5):423-31.

PMID:
14518824
7.

Medical aspects of ketone body metabolism.

Mitchell GA, Kassovska-Bratinova S, Boukaftane Y, Robert MF, Wang SP, Ashmarina L, Lambert M, Lapierre P, Potier E.

Clin Invest Med. 1995 Jun;18(3):193-216. Review.

PMID:
7554586
8.

Patients homozygous for the T435N mutation of succinyl-CoA:3-ketoacid CoA Transferase (SCOT) do not show permanent ketosis.

Fukao T, Shintaku H, Kusubae R, Zhang GX, Nakamura K, Kondo M, Kondo N.

Pediatr Res. 2004 Dec;56(6):858-63.

PMID:
15496607
9.

Impact of peripheral ketolytic deficiency on hepatic ketogenesis and gluconeogenesis during the transition to birth.

Cotter DG, Ercal B, d'Avignon DA, Dietzen DJ, Crawford PA.

J Biol Chem. 2013 Jul 5;288(27):19739-49. doi: 10.1074/jbc.M113.454868.

10.

Neonatal hypoglycaemia in severe succinyl-CoA: 3-oxoacid CoA-transferase deficiency.

Berry GT, Fukao T, Mitchell GA, Mazur A, Ciafre M, Gibson J, Kondo N, Palmieri MJ.

J Inherit Metab Dis. 2001 Oct;24(5):587-95.

PMID:
11757586
11.

Clinical and molecular characterization of five patients with succinyl-CoA:3-ketoacid CoA transferase (SCOT) deficiency.

Fukao T, Sass JO, Kursula P, Thimm E, Wendel U, Ficicioglu C, Monastiri K, Guffon N, Barić I, Zabot MT, Kondo N.

Biochim Biophys Acta. 2011 May;1812(5):619-24. doi: 10.1016/j.bbadis.2011.01.015.

12.

Hyperketotic states due to inherited defects of ketolysis.

Saudubray JM, Specola N, Middleton B, Lombes A, Bonnefont JP, Jakobs C, Vassault A, Charpentier C, Day R.

Enzyme. 1987;38(1-4):80-90. Review.

PMID:
2894307
13.

Obligate role for ketone body oxidation in neonatal metabolic homeostasis.

Cotter DG, d'Avignon DA, Wentz AE, Weber ML, Crawford PA.

J Biol Chem. 2011 Mar 4;286(9):6902-10. doi: 10.1074/jbc.M110.192369.

14.

Inborn errors of ketogenesis and ketone body utilization.

Sass JO.

J Inherit Metab Dis. 2012 Jan;35(1):23-8. doi: 10.1007/s10545-011-9324-6. Review.

PMID:
21479626
15.

Fatty acid and ketone body metabolism in the rat: response to diet and exercise.

Askew EW, Dohm GL, Huston RL.

J Nutr. 1975 Nov;105(11):1422-32.

PMID:
475
16.

Prenatal diagnosis of succinyl-coenzyme A:3-ketoacid coenzyme A transferase deficiency.

Fukao T, Song XQ, Watanabe H, Hirayama K, Sakazaki H, Shintaku H, Imanaka M, Orii T, Kondo N.

Prenat Diagn. 1996 May;16(5):471-4.

PMID:
8844009
17.

Differential utilization of ketone bodies by neurons and glioma cell lines: a rationale for ketogenic diet as experimental glioma therapy.

Maurer GD, Brucker DP, Bähr O, Harter PN, Hattingen E, Walenta S, Mueller-Klieser W, Steinbach JP, Rieger J.

BMC Cancer. 2011 Jul 26;11:315. doi: 10.1186/1471-2407-11-315.

18.

A structural mapping of mutations causing succinyl-CoA:3-ketoacid CoA transferase (SCOT) deficiency.

Shafqat N, Kavanagh KL, Sass JO, Christensen E, Fukao T, Lee WH, Oppermann U, Yue WW.

J Inherit Metab Dis. 2013 Nov;36(6):983-7. doi: 10.1007/s10545-013-9589-z.

19.

Succinyl-CoA:3-ketoacid coenzyme A transferase (SCOT): development of an antibody to human SCOT and diagnostic use in hereditary SCOT deficiency.

Song XQ, Fukao T, Mitchell GA, Kassovska-Bratinova S, Ugarte M, Wanders RJ, Hirayama K, Shintaku H, Churchill P, Watanabe H, Orii T, Kondo N.

Biochim Biophys Acta. 1997 Apr 12;1360(2):151-6.

PMID:
9128180
20.

Developmental changes in ketogenic enzyme gene expression during sheep rumen development.

Lane MA, Baldwin RL 4th, Jesse BW.

J Anim Sci. 2002 Jun;80(6):1538-44.

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