메뉴 건너뛰기




Volumn 11, Issue 3, 2011, Pages 533-536

Fatal heart failure associated with CoQ10 and multiple OXPHOS deficiency in a child with propionic acidemia

Author keywords

CoQ10 deficiency; Mitochondrial disorders; Propionic acidemia

Indexed keywords

CITRATE SYNTHASE; CITRIC ACID; GLYCINE; METRONIDAZOLE; PROPIONIC ACID; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE DEHYDROGENASE (UBIQUINONE); SUCCINATE DEHYDROGENASE (UBIQUINONE); UBIDECARENONE; UBIQUINOL CYTOCHROME C REDUCTASE; UBIQUINONE;

EID: 79953758676     PISSN: 15677249     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.mito.2011.02.002     Document Type: Article
Times cited : (37)

References (26)
  • 1
    • 0017184389 scopus 로고
    • A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding
    • Bradford M. A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding. Annal. Biochem. 1976, 72:248-254.
    • (1976) Annal. Biochem. , vol.72 , pp. 248-254
    • Bradford, M.1
  • 2
    • 3442889456 scopus 로고    scopus 로고
    • On the mechanism of action of the antifungal agent propionate
    • Brock M., Buckel W. On the mechanism of action of the antifungal agent propionate. Eur. J. Biochem. 2004, 271:3727-3741.
    • (2004) Eur. J. Biochem. , vol.271 , pp. 3727-3741
    • Brock, M.1    Buckel, W.2
  • 4
    • 0016717991 scopus 로고
    • Effect of 2-methylcitrate on citrate metabolism: implications for the management of patients with propionic acidemia and methylmalonic aciduria
    • Cheema-Dhadii S., Leznoff C., Halperin M. Effect of 2-methylcitrate on citrate metabolism: implications for the management of patients with propionic acidemia and methylmalonic aciduria. Pediatr. Res. 1975, 9:905-908.
    • (1975) Pediatr. Res. , vol.9 , pp. 905-908
    • Cheema-Dhadii, S.1    Leznoff, C.2    Halperin, M.3
  • 7
    • 0019441244 scopus 로고
    • The specific inhibition of thepyruvate dehydrogenase complex from pig kidney in propionyl-CoA and isovaleryl-CoA
    • Gregersen N. The specific inhibition of thepyruvate dehydrogenase complex from pig kidney in propionyl-CoA and isovaleryl-CoA. Biochem. Med. 1981, 26:20-27.
    • (1981) Biochem. Med. , vol.26 , pp. 20-27
    • Gregersen, N.1
  • 8
    • 0035374693 scopus 로고    scopus 로고
    • Studies of propionate toxicity in Salmonella Enterica identify 2-methylcitrate as a potent inhibitor of cell growth
    • Horswill A., Dudding A., Escalante-Semerena J. Studies of propionate toxicity in Salmonella Enterica identify 2-methylcitrate as a potent inhibitor of cell growth. J. Biol. Chem. 2001, 276:19094-19101.
    • (2001) J. Biol. Chem. , vol.276 , pp. 19094-19101
    • Horswill, A.1    Dudding, A.2    Escalante-Semerena, J.3
  • 13
    • 23044475283 scopus 로고    scopus 로고
    • Clinical, pathological, and biochemical studies in a patient with propionic acidemia and fatal cardiomyopathy
    • Mardach R., Anthony Verity M., Cederbaum S. Clinical, pathological, and biochemical studies in a patient with propionic acidemia and fatal cardiomyopathy. Mol. Genet. Metab. 2005, 85:286-290.
    • (2005) Mol. Genet. Metab. , vol.85 , pp. 286-290
    • Mardach, R.1    Anthony Verity, M.2    Cederbaum, S.3
  • 14
    • 0027233542 scopus 로고
    • Cardiomyopathy in propionic acidaemia
    • Massoud A., Leonard J. Cardiomyopathy in propionic acidaemia. Eur. J. Pediatr. 1993, 152:441-445.
    • (1993) Eur. J. Pediatr. , vol.152 , pp. 441-445
    • Massoud, A.1    Leonard, J.2
  • 15
    • 67749117788 scopus 로고    scopus 로고
    • Profiling of oxidative stress in patients with inborn errors of metabolism
    • Mc Guire P., Parikh A., Diaz G. Profiling of oxidative stress in patients with inborn errors of metabolism. Mol. Genet. Metab. 2009, 98:173-180.
    • (2009) Mol. Genet. Metab. , vol.98 , pp. 173-180
    • Mc Guire, P.1    Parikh, A.2    Diaz, G.3
  • 21
    • 34248151929 scopus 로고    scopus 로고
    • Infantile and pediatric quinone deficiency diseases
    • Rotig A., Mollet J., Rio M., Munnich A. Infantile and pediatric quinone deficiency diseases. Mitochondrion 2007, 7:S212-S221.
    • (2007) Mitochondrion , vol.7
    • Rotig, A.1    Mollet, J.2    Rio, M.3    Munnich, A.4


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.