-
1
-
-
0000576457
-
Mitochondrial fatty acid oxidation disorders
-
Scriver CR, Beaudet AL, Sly WS, Valle D. New York: McGraw-Hill
-
Roe CR, Coates PM. Mitochondrial fatty acid oxidation disorders. In: Scriver CR, Beaudet AL, Sly WS, Valle D. The metabolic and molecular bases of inherited disease. 7th ed. Vol. 1. New York: McGraw-Hill, 1995:1501-33.
-
(1995)
The Metabolic and Molecular Bases of Inherited Disease. 7th Ed.
, vol.1
, pp. 1501-1533
-
-
Roe, C.R.1
Coates, P.M.2
-
2
-
-
0026549217
-
Regulation of the expression of the mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase gene: Its role in the control of ketogenesis
-
Casals N, Roca N, Guerrero M, et al. Regulation of the expression of the mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase gene: its role in the control of ketogenesis. Biochem J 1992;283:261-4.
-
(1992)
Biochem J
, vol.283
, pp. 261-264
-
-
Casals, N.1
Roca, N.2
Guerrero, M.3
-
3
-
-
0027429339
-
Methylation of the regulatory region of the mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase gene leads to its transcriptional inactivation
-
Ayte J, Gil-Gomez G, Hegardt FG. Methylation of the regulatory region of the mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase gene leads to its transcriptional inactivation. Biochem J 1993;295:807-12.
-
(1993)
Biochem J
, vol.295
, pp. 807-812
-
-
Ayte, J.1
Gil-Gomez, G.2
Hegardt, F.G.3
-
4
-
-
0027365446
-
Regulation of mitochondrial 3-hydroxy-3-methylglutaryl-coenzyme a synthase protein by starvation, fat feeding, and diabetes
-
Serra D, Casals N, Asins G, Royo T, Ciudad CJ, Hegardt FG. Regulation of mitochondrial 3-hydroxy-3-methylglutaryl-coenzyme A synthase protein by starvation, fat feeding, and diabetes. Arch Biochem Biophys 1993;307:40-5.
-
(1993)
Arch Biochem Biophys
, vol.307
, pp. 40-45
-
-
Serra, D.1
Casals, N.2
Asins, G.3
Royo, T.4
Ciudad, C.J.5
Hegardt, F.G.6
-
5
-
-
0025352338
-
Rat mitochondrial and cytosolic 3-hydroxy-3-methylglutaryl-CoA synthases are encoded by two different genes
-
Ayte J, Gil-Gomez G, Haro D, Marrero PF, Hegardt FG. Rat mitochondrial and cytosolic 3-hydroxy-3-methylglutaryl-CoA synthases are encoded by two different genes. Proc Natl Acad Sci U S A 1990;87:3874-8.
-
(1990)
Proc Natl Acad Sci U S A
, vol.87
, pp. 3874-3878
-
-
Ayte, J.1
Gil-Gomez, G.2
Haro, D.3
Marrero, P.F.4
Hegardt, F.G.5
-
7
-
-
0017104334
-
3-Hydroxy-3-methylglutaric aciduria: 3-hydroxy-3-methylglutaryl-coenzyme a lyase levels in leucocytes
-
Wysocki SJ, Hahnel R. 3-Hydroxy-3-methylglutaric aciduria: 3-hydroxy-3-methylglutaryl-coenzyme A lyase levels in leucocytes. Clin Chim Acta 1976;73:373-5.
-
(1976)
Clin Chim Acta
, vol.73
, pp. 373-375
-
-
Wysocki, S.J.1
Hahnel, R.2
-
8
-
-
0015305639
-
A method for the determination of carnitine in the picomole range
-
Cederblad G, Lindstedt S. A method for the determination of carnitine in the picomole range. Clin Chim Acta 1972;37:235-43.
-
(1972)
Clin Chim Acta
, vol.37
, pp. 235-243
-
-
Cederblad, G.1
Lindstedt, S.2
-
9
-
-
1842354872
-
Miniaturisation et automatization du dosage du glucose, du pyruvate, du lactate, de l'acetoacetate, du β-hydroxybutyrate, des acides gras nonesterifies sanguins
-
Fourré C, Rota M, Vassault A, Bonnefont JP, Nicolas A, Bailly M. Miniaturisation et automatization du dosage du glucose, du pyruvate, du lactate, de l'acetoacetate, du β-hydroxybutyrate, des acides gras nonesterifies sanguins. Acta Pharm Biol Clin 1987;4:420-3.
-
(1987)
Acta Pharm Biol Clin
, vol.4
, pp. 420-423
-
-
Fourré, C.1
Rota, M.2
Vassault, A.3
Bonnefont, J.P.4
Nicolas, A.5
Bailly, M.6
-
10
-
-
0001222859
-
The analysis of diagnostic markers of genetic disorders in human blood and urine using tandem mass spectrometry with liquid SIMS
-
Millington DS, Kodo N, Terada N, Roe D, Chace DH. The analysis of diagnostic markers of genetic disorders in human blood and urine using tandem mass spectrometry with liquid SIMS. Int J Mass Spectrom 1991; 111:211-6.
-
(1991)
Int J Mass Spectrom
, vol.111
, pp. 211-216
-
-
Millington, D.S.1
Kodo, N.2
Terada, N.3
Roe, D.4
Chace, D.H.5
-
11
-
-
0023919487
-
Cis-4-decenoic acid in plasma: A characteristic metabolite in medium-chain acylCoa dehydrogenase deficiency
-
Duran M, Bruinvis L, Ketting D, de Klerk JBC, Wadman SK. Cis-4-decenoic acid in plasma: a characteristic metabolite in medium-chain acylCoA dehydrogenase deficiency. Clin Chem 1988;34:548-51.
-
(1988)
Clin Chem
, vol.34
, pp. 548-551
-
-
Duran, M.1
Bruinvis, L.2
Ketting, D.3
De Klerk, J.B.C.4
Wadman, S.K.5
-
12
-
-
0019174319
-
Gas-chromatographic method of analysis for urinary organic acids. II. Description of the procedure, and its application to diagnosis of patients with organic acidurias
-
Tanaka K, West-Dull A, Hine DG, Lynn TB, Lowe T. Gas-chromatographic method of analysis for urinary organic acids. II. Description of the procedure, and its application to diagnosis of patients with organic acidurias. Clin Chem 1980;26:1847-53.
-
(1980)
Clin Chem
, vol.26
, pp. 1847-1853
-
-
Tanaka, K.1
West-Dull, A.2
Hine, D.G.3
Lynn, T.B.4
Lowe, T.5
-
14
-
-
0020681655
-
Development of hepatic fatty acid oxidation and ketogenesis in the newborn guinea pig
-
Stanley CA, Gonzales E, Baker L. Development of hepatic fatty acid oxidation and ketogenesis in the newborn guinea pig. Pediatr Res 1983;17: 224-9.
-
(1983)
Pediatr Res
, vol.17
, pp. 224-229
-
-
Stanley, C.A.1
Gonzales, E.2
Baker, L.3
-
15
-
-
0014303713
-
Activity and intracellular distribution of enzymes of ketone-body metabolism in rat liver
-
Williamson DH, Bates MW, Krebs HA. Activity and intracellular distribution of enzymes of ketone-body metabolism in rat liver. Biochem J 1968;108:353-61.
-
(1968)
Biochem J
, vol.108
, pp. 353-361
-
-
Williamson, D.H.1
Bates, M.W.2
Krebs, H.A.3
-
16
-
-
0026703357
-
A deficiency of carnitine-acylcarnitine translocase in the inner mitochondrial membrane
-
Stanley CA, Hale DE, Berry GT, Deleeuw S, Boxer J, Bonnefont J-P. A deficiency of carnitine-acylcarnitine translocase in the inner mitochondrial membrane. N Engl J Med 1992;327:19-23.
-
(1992)
N Engl J Med
, vol.327
, pp. 19-23
-
-
Stanley, C.A.1
Hale, D.E.2
Berry, G.T.3
Deleeuw, S.4
Boxer, J.5
Bonnefont, J.-P.6
-
17
-
-
0024451319
-
Treatment of rats with glucagon or mannoheptulose increases mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase activity and decreases succinyl-CoA content in liver
-
Quant PA, Tubbs PK, Brand MD. Treatment of rats with glucagon or mannoheptulose increases mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase activity and decreases succinyl-CoA content in liver. Biochem J 1989;262:159-64.
-
(1989)
Biochem J
, vol.262
, pp. 159-164
-
-
Quant, P.A.1
Tubbs, P.K.2
Brand, M.D.3
-
18
-
-
0027252589
-
Regulation of fatty acid oxidation in mammalian liver
-
Guzman M, Geelen MJ. Regulation of fatty acid oxidation in mammalian liver. Biochim Biophys Acta 1993;1167:227-41.
-
(1993)
Biochim Biophys Acta
, vol.1167
, pp. 227-241
-
-
Guzman, M.1
Geelen, M.J.2
-
19
-
-
0023906977
-
Role of carnitine palmitoyltransrerase I in the regulation of hepatic ketogenesis during the onset and reversal of chronic diabetes
-
Grantham BD, Zammit VA. Role of carnitine palmitoyltransrerase I in the regulation of hepatic ketogenesis during the onset and reversal of chronic diabetes. Biochem J 1988;249:409-14.
-
(1988)
Biochem J
, vol.249
, pp. 409-414
-
-
Grantham, B.D.1
Zammit, V.A.2
-
20
-
-
0027190037
-
The rat mitochondrial 3-hydroxy-3-methylglutaryl-coenzyme-A-synthase gene contains elements that mediate its multihormonal regulation and tissue specificity
-
Gil Gomez G, Ayte J, Hegardt FG. The rat mitochondrial 3-hydroxy-3-methylglutaryl-coenzyme-A-synthase gene contains elements that mediate its multihormonal regulation and tissue specificity. Eur J Biochem 1993;213:773-9.
-
(1993)
Eur J Biochem
, vol.213
, pp. 773-779
-
-
Gil Gomez, G.1
Ayte, J.2
Hegardt, F.G.3
-
21
-
-
0025633304
-
Avian liver 3-hydroxy-3-methylglutaryl-CoA synthase: Distinct genes encode the cholesterogenic and ketogenic isozymes
-
Kattar-Cooley PA, Wang HH, Mende-Mueller LM, Miziorko HM. Avian liver 3-hydroxy-3-methylglutaryl-CoA synthase: distinct genes encode the cholesterogenic and ketogenic isozymes. Arch Biochem Biophys 1990;283:523-9.
-
(1990)
Arch Biochem Biophys
, vol.283
, pp. 523-529
-
-
Kattar-Cooley, P.A.1
Wang, H.H.2
Mende-Mueller, L.M.3
Miziorko, H.M.4
-
22
-
-
0024329415
-
Formation of ketone bodies by resting lymphocytes
-
Curi R, Williams JF, Newsholme EA. Formation of ketone bodies by resting lymphocytes. Int J Biochem 1989;21:1133-6.
-
(1989)
Int J Biochem
, vol.21
, pp. 1133-1136
-
-
Curi, R.1
Williams, J.F.2
Newsholme, E.A.3
-
23
-
-
0027319703
-
Testis and ovary express the gene for the ketogenic mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase
-
Royo T, Pedragosa MJ, Ayte J, Gil-Gomez G, Vilaro S, Hegardt FG. Testis and ovary express the gene for the ketogenic mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase. J Lipid Res 1993;34:1636.
-
(1993)
J Lipid Res
, vol.34
, pp. 1636
-
-
Royo, T.1
Pedragosa, M.J.2
Ayte, J.3
Gil-Gomez, G.4
Vilaro, S.5
Hegardt, F.G.6
-
24
-
-
0025099536
-
The contribution of protein catabolism to metabolic decompensation in 3-hydroxy-3-methylglutaric aciduria
-
Thompson GN, Chalmers RA, Halliday D. The contribution of protein catabolism to metabolic decompensation in 3-hydroxy-3-methylglutaric aciduria. Eur J Pediatr 1990;149:346-50.
-
(1990)
Eur J Pediatr
, vol.149
, pp. 346-350
-
-
Thompson, G.N.1
Chalmers, R.A.2
Halliday, D.3
-
25
-
-
0021884157
-
Ketone body metabolism during development
-
Williamson DH. Ketone body metabolism during development. Fed Proc 1985;44:2342-6.
-
(1985)
Fed Proc
, vol.44
, pp. 2342-2346
-
-
Williamson, D.H.1
|