메뉴 건너뛰기




Volumn 29, Issue 4, 1991, Pages 406-411

Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency

Author keywords

[No Author keywords available]

Indexed keywords

3 HYDROXYACYL COENZYME A DEHYDROGENASE;

EID: 0025868680     PISSN: 00313998     EISSN: 15300447     Source Type: Journal    
DOI: 10.1203/00006450-199104000-00016     Document Type: Article
Times cited : (94)

References (22)
  • 6
    • 0038275335 scopus 로고
    • Deficiency of electron transfer flavoprotein or electron transfer flavoprotein ubiquinone oxidoreductase in glutaric aciduria type II fibroblasts
    • Frerman FE, Goodman SI 1985 Deficiency of electron transfer flavoprotein or electron transfer flavoprotein ubiquinone oxidoreductase in glutaric aciduria type II fibroblasts. Proc Natl Acad Sci USA 82:4517-4520
    • (1985) Proc Natl Acad Sci USA , vol.82 , pp. 4517-4520
    • Frerman, F.E.1    Goodman, S.I.2
  • 7
    • 0020481368 scopus 로고
    • The existence of an inner-membrane-bound, long acyl-chain-specific 3-hydroxyacyI-CoA dehydrogenase in mammalian mitochondria
    • El Fakhri M, Middleton B 1982 The existence of an inner-membrane-bound, long acyl-chain-specific 3-hydroxyacyI-CoA dehydrogenase in mammalian mitochondria. Biochim Biophys Acta 713:270-279
    • (1982) Biochim Biophys Acta , vol.713 , pp. 270-279
    • El Fakhri, M.1    Middleton, B.2
  • 8
    • 0024353075 scopus 로고
    • Sudden infant death and long-chain 3-hydroxyacyl-CoA dehydrogenase
    • Wanders RJA, Duran M, Ijlst L, de Jager JP 1989 Sudden infant death and long-chain 3-hydroxyacyl-CoA dehydrogenase. Lancet 2:52-53
    • (1989) Lancet , vol.2 , pp. 52-53
    • Wanders, R.1    Duran, M.2    Ijlst, L.3    De Jager, J.P.4
  • 11
    • 0025138203 scopus 로고
    • Clinical and biochemical presentations in 20 cases of hydrox-ydicarboxylic aciduria
    • Tanaka K, Coates PM (eds), Alan R Liss Inc, New York
    • Pollitt RJ 1990 Clinical and biochemical presentations in 20 cases of hydrox-ydicarboxylic aciduria. In: Tanaka K, Coates PM (eds) Fatty Acid Oxidation: Clinical, Biochemical and Molecular Aspects. Alan R Liss Inc, New York, pp 495-502
    • (1990) Fatty Acid Oxidation: Clinical, Biochemical and Molecular Aspects , pp. 495-502
    • Pollitt, R.J.1
  • 12
    • 85039300494 scopus 로고
    • An analysis of partial reactions in the overall chain elongation of saturated and unsaturated fatty acids by rat liver microsomes
    • Benert JT, Sprecher H 1977 An analysis of partial reactions in the overall chain elongation of saturated and unsaturated fatty acids by rat liver microsomes. J Biol Chem 25:391-394
    • (1977) J Biol Chem , vol.25 , pp. 391-394
    • Benert, J.T.1    Sprecher, H.2
  • 13
    • 0024463077 scopus 로고
    • Measurement of intact acyl-CoA intermediates of β-oxidation by h.P.l.c. with on-line radiochemical and photodiodearray detection: Application to the study of [U-,4C]-hexadecanoate oxidation by rat liver mitochondria
    • Watmough NJ, Turnbull DM, Sherratt HSA, Bartlett K 1989 Measurement of intact acyl-CoA intermediates of /3-oxidation by h.p.l.c. with on-line radiochemical and photodiodearray detection: application to the study of [U-,4C]-hexadecanoate oxidation by rat liver mitochondria. Biochem J 262:261-269
    • (1989) Biochem J , vol.262 , pp. 261-269
    • Watmough, N.J.1    Turnbull, D.M.2    Sherratt, H.3    Bartlett, K.4
  • 14
    • 0022728214 scopus 로고
    • A method for the preparation of 3-ketoacyl-CoA derivatives
    • Thorpe C 1986 A method for the preparation of 3-ketoacyl-CoA derivatives. Anal Biochem 155:391-394
    • (1986) Anal Biochem , vol.155 , pp. 391-394
    • Thorpe, C.1
  • 15
    • 0019343809 scopus 로고
    • Short-chain and long-chain enoyl-CoA hydratascs from pig heart muscle
    • Fong JC, Schulz H 1981 Short-chain and long-chain enoyl-CoA hydratascs from pig heart muscle. Methods Enzymol 71:390-398
    • (1981) Methods Enzymol , vol.71 , pp. 390-398
    • Fong, J.C.1    Schulz, H.2
  • 16
    • 0017800480 scopus 로고
    • Purification and properties of a pig heart thiolase with broad chain length specificity and comparison of thiolases from pig heart and Escherichia coli
    • Staack H, Binstock JF, Schulz H 1978 Purification and properties of a pig heart thiolase with broad chain length specificity and comparison of thiolases from pig heart and Escherichia coli. J Biol Chem 253:1827-1831
    • (1978) J Biol Chem , vol.253 , pp. 1827-1831
    • Staack, H.1    Binstock, J.F.2    Schulz, H.3
  • 17
    • 0015530438 scopus 로고
    • The existence of ketoacyl-CoA thiolases of differing properties and intracellular localization in ox liver
    • Middleton B 1972 The existence of ketoacyl-CoA thiolases of differing properties and intracellular localization in ox liver. Biochem Biophys Res Commun 46:508-515
    • (1972) Biochem Biophys Res Commun , vol.46 , pp. 508-515
    • Middleton, B.1
  • 18
    • 0021943592 scopus 로고
    • Fluorometric assay of acyl-CoA dehydrogen-ases in normal and mutant human fibroblasts
    • Frerman FE, Goodman SI 1985 Fluorometric assay of acyl-CoA dehydrogen-ases in normal and mutant human fibroblasts. Biochem Med 33:38-44
    • (1985) Biochem Med , vol.33 , pp. 38-44
    • Frerman, F.E.1    Goodman, S.I.2
  • 20
    • 2042510688 scopus 로고
    • Inherited disorders of mitochondrial ß-oxidation
    • Schob J. Van Hoof F, Vis HL (eds), Inborn Errors of Metabolism. Vevcy Raven Press, Ltd, New York
    • Bartlett K, Anyslcy-Green A, Leonard JV, Turnbull DM 1990 Inherited disorders of mitochondrial ß-oxidation. In: Schob J. Van Hoof F, Vis HL (eds) Nestlé Nutrition Workshop Series, Vol 24, Inborn Errors of Metabolism. Vevcy Raven Press, Ltd, New York, pp 19-40
    • (1990) Nestlé Nutrition Workshop Series , vol.24 , pp. 19-40
    • Bartlett, K.1    Anyslcy-Green, A.2    Leonard, J.V.3    Turnbull, D.M.4
  • 21
    • 0025285225 scopus 로고
    • Isolation of a malonyl-CoA-sensitive CPT/β-oxidation enzyme complex from heart mitochondria
    • Kcrner J, Bieber L 1990 Isolation of a malonyl-CoA-sensitive CPT/β-oxidation enzyme complex from heart mitochondria. Biochemistry 29:4326-4334
    • (1990) Biochemistry , vol.29 , pp. 4326-4334
    • Kcrner, J.1    Bieber, L.2
  • 22
    • 0020540868 scopus 로고
    • Hypoglycaemia, hepatic dysfunction, muscle weakness, cardiomyopathy, free carnitine deficiency and long-chain acylcarnitine excess responsive to medium chain triglyceride diet
    • Glasgow AM, Engel AG, Bier DM, Perry LW, Dickie M, Todaro J, Brown BI, Utter MF 1983 Hypoglycaemia, hepatic dysfunction, muscle weakness, cardiomyopathy, free carnitine deficiency and long-chain acylcarnitine excess responsive to medium chain triglyceride diet. Pediatr Res 17:319-323
    • (1983) Pediatr Res , vol.17 , pp. 319-323
    • Glasgow, A.M.1    Engel, A.G.2    Bier, D.M.3    Perry, L.W.4    Dickie, M.5    Todaro, J.6    Brown, B.I.7    Utter, M.F.8


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.