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Volumn 4, Issue 5-6 SPEC. ISS., 2004, Pages 387-394

Respiratory chain analysis of skin fibroblasts in mitochondrial disease

Author keywords

Leigh syndrome; Mitochondria; Respiratory chain

Indexed keywords

CARBON 14; LACTIC ACID; PYRUVATE OXIDASE;

EID: 10644226829     PISSN: 15677249     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.mito.2004.07.039     Document Type: Article
Times cited : (23)

References (10)
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    • Revised assays for the investigation of congenital lactic acidosis using 14C keto acids, eliminating problems associated with spontaneous decarboxylation
    • K. Hyland J.V. Leonard Revised assays for the investigation of congenital lactic acidosis using 14C keto acids, eliminating problems associated with spontaneous decarboxylation Clin. Chim. Acta 133 1983 177-187
    • (1983) Clin. Chim. Acta , vol.133 , pp. 177-187
    • Hyland, K.1    Leonard, J.V.2
  • 5
    • 0021162081 scopus 로고
    • Deficiency of the iron-sulfur clusters of mitochondrial reduced nicotinamide-adenine dinucleotide-ubiquinone oxidoreductase (complex I) in an infant with congenital lactic acidosis
    • R.W. Moreadith M.L. Batshaw T. Ohnishi D. Kerr B. Knox D. Jackson R. Hruban J. Olson B. Reynafarje A.L. Lehninger Deficiency of the iron-sulfur clusters of mitochondrial reduced nicotinamide-adenine dinucleotide-ubiquinone oxidoreductase (complex I) in an infant with congenital lactic acidosis J. Clin. Invest. 74 1984 685-697
    • (1984) J. Clin. Invest. , vol.74 , pp. 685-697
    • Moreadith, R.W.1    Batshaw, M.L.2    Ohnishi, T.3    Kerr, D.4    Knox, B.5    Jackson, D.6    Hruban, R.7    Olson, J.8    Reynafarje, B.9    Lehninger, A.L.10
  • 6
    • 0030056515 scopus 로고    scopus 로고
    • Mitochondrial complex I deficiency leads to increased production of superoxide radicals and induction of superoxide dismutase
    • S. Pitkanen B.H. Robinson Mitochondrial complex I deficiency leads to increased production of superoxide radicals and induction of superoxide dismutase J. Clin. Invest. 98 1996 345-351
    • (1996) J. Clin. Invest. , vol.98 , pp. 345-351
    • Pitkanen, S.1    Robinson, B.H.2
  • 7
    • 0030447468 scopus 로고    scopus 로고
    • Diagnosis of complex I deficiency in patients with lactic acidemia using skin fibroblast cultures
    • S. Pitkanen S. Raha B.H. Robinson Diagnosis of complex I deficiency in patients with lactic acidemia using skin fibroblast cultures Biochem. Mol. Med. 59 1996 134-137
    • (1996) Biochem. Mol. Med. , vol.59 , pp. 134-137
    • Pitkanen, S.1    Raha, S.2    Robinson, B.H.3
  • 8
    • 0029874964 scopus 로고    scopus 로고
    • Use of fibroblast and lymphoblast cultures for detection of respiratory chain defects
    • B.H. Robinson Use of fibroblast and lymphoblast cultures for detection of respiratory chain defects Methods Enzymol. 264 1996 454-464
    • (1996) Methods Enzymol. , vol.264 , pp. 454-464
    • Robinson, B.H.1
  • 9
    • 0020646525 scopus 로고
    • Lactic acidosis, neurological deterioration and compromised cellular pyruvate oxidation due to a defect in the reoxidation of cytoplasmically generated NADH
    • B.H. Robinson J. Taylor B. Francois A.L. Beaudet D.F. Peterson Lactic acidosis, neurological deterioration and compromised cellular pyruvate oxidation due to a defect in the reoxidation of cytoplasmically generated NADH Eur. J. Pediatr. 140 1983 98-101
    • (1983) Eur. J. Pediatr. , vol.140 , pp. 98-101
    • Robinson, B.H.1    Taylor, J.2    Francois, B.3    Beaudet, A.L.4    Peterson, D.F.5
  • 10
    • 0025123850 scopus 로고
    • The use of skin fibroblast cultures in the detection of respiratory chain defects in patients with lacticacidemia
    • B.H. Robinson D.M. Glerum W. Chow R. Petrova-Benedict R. Lightowlers R. Capaldi The use of skin fibroblast cultures in the detection of respiratory chain defects in patients with lacticacidemia Pediatr. Res. 28 1990 549-555
    • (1990) Pediatr. Res. , vol.28 , pp. 549-555
    • Robinson, B.H.1    Glerum, D.M.2    Chow, W.3    Petrova-Benedict, R.4    Lightowlers, R.5    Capaldi, R.6


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.