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Volumn 19, Issue 3, 1996, Pages 296-300

Two siblings with episodic ketoacidosis and decreased activity of succinyl-CoA:3-ketoacid CoA-transferase in cultured fibroblasts

Author keywords

[No Author keywords available]

Indexed keywords

3 HYDROXYBUTYRIC ACID; 3 OXOACID COENZYME A TRANSFERASE; ACETOACETIC ACID; SALICYLIC ACID;

EID: 0029993142     PISSN: 01418955     EISSN: None     Source Type: Journal    
DOI: 10.1007/BF01799257     Document Type: Article
Times cited : (24)

References (11)
  • 3
    • 0001000590 scopus 로고
    • Infantile ketoacidosis associated with decreased activity of succinyl-CoA:3-ketoacid CoA-transferase
    • Middleton B, Day R, Lombes A, Saudubray JM (1987) Infantile ketoacidosis associated with decreased activity of succinyl-CoA:3-ketoacid CoA-transferase. J Inher Metab Dis 10(supplement 2): 273-275.
    • (1987) J Inher Metab Dis , vol.10 , Issue.2 SUPPL. , pp. 273-275
    • Middleton, B.1    Day, R.2    Lombes, A.3    Saudubray, J.M.4
  • 5
    • 58149407489 scopus 로고
    • Acetoacetyl-CoA thiolase deficiency: A cause of severe acidosis in infancy simulating salicylism
    • Robinson BH, Sherwood WG, Taylor J, Balfe W, Marner OA (1979) Acetoacetyl-CoA thiolase deficiency: a cause of severe acidosis in infancy simulating salicylism. J Pediatrics 95: 228-233.
    • (1979) J Pediatrics , vol.95 , pp. 228-233
    • Robinson, B.H.1    Sherwood, W.G.2    Taylor, J.3    Balfe, W.4    Marner, O.A.5
  • 6
    • 0029117717 scopus 로고
    • A new Japanese case of succinyl-CoA:3-ketoacid CoA-transferase deficiency
    • Sakazaki H, Hiryama K, Murakami S, et al (1995) A new Japanese case of succinyl-CoA:3-ketoacid CoA-transferase deficiency. J Inher Metab Dis 18: 323-325.
    • (1995) J Inher Metab Dis , vol.18 , pp. 323-325
    • Sakazaki, H.1    Hiryama, K.2    Murakami, S.3
  • 7
    • 0023472290 scopus 로고
    • Hyperketotic states due to inherited defects of ketolysis
    • Saudubray JM, Specola N, Middleton B, et al (1987) Hyperketotic states due to inherited defects of ketolysis. Enzyme 38: 80-90.
    • (1987) Enzyme , vol.38 , pp. 80-90
    • Saudubray, J.M.1    Specola, N.2    Middleton, B.3
  • 9
    • 0015310967 scopus 로고
    • Succinyl-CoA:3-ketoacid-CoA transferase deficiency as a cause for ketoacidosis in infancy
    • Tildon JT, Cornblath M (1972) Succinyl-CoA:3-ketoacid-CoA transferase deficiency as a cause for ketoacidosis in infancy. J Clin Invest 51: 493-498.
    • (1972) J Clin Invest , vol.51 , pp. 493-498
    • Tildon, J.T.1    Cornblath, M.2
  • 10
    • 0014972144 scopus 로고
    • Activities of enzymes involved in acetoacetate utilization in adult mammalian tissues
    • Williamson DH, Bates MW, Page MA, Krebs HA (1971) Activities of enzymes involved in acetoacetate utilization in adult mammalian tissues. Biochem J 121: 41-47.
    • (1971) Biochem J , vol.121 , pp. 41-47
    • Williamson, D.H.1    Bates, M.W.2    Page, M.A.3    Krebs, H.A.4
  • 11
    • 0027159208 scopus 로고
    • Biochemical and immunochemical study of seven families with 3-ketothiolase deficiency: Diagnosis using immunochemical determination of the ratio of mitochondrial acetoacetyl-CoA thiolase and 3-ketoacyl-CoA thiolase proteins
    • Yamaguchi S, Sakai A, Fukao T et al (1993) Biochemical and immunochemical study of seven families with 3-ketothiolase deficiency: diagnosis using immunochemical determination of the ratio of mitochondrial acetoacetyl-CoA thiolase and 3-ketoacyl-CoA thiolase proteins. Pediatr Res 33: 429-432.
    • (1993) Pediatr Res , vol.33 , pp. 429-432
    • Yamaguchi, S.1    Sakai, A.2    Fukao, T.3


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.