Effect of various agents on adenosine triphosphate synthesis in mitochondrial complex I deficiency

Journal of Pediatrics

Volumn 139, Issue 6, 2001, Pages 868-870

  Bar Meir, Maskit ^a   Elpeleg, Orly N ^a   Saada, Ann ^a  

^a SHAARE ZEDEK MEDICAL CENTER   (Israel)

Author keywords

[No Author keywords available]

Indexed keywords

ADENOSINE TRIPHOSPHATE; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE DEHYDROGENASE (UBIQUINONE); RIBOFLAVIN;

ARTICLE; CASE REPORT; COMPUTER ASSISTED TOMOGRAPHY; DISORDERS OF MITOCHONDRIAL FUNCTIONS; ENZYME DEFICIENCY; FIBROBLAST; HUMAN; HUMAN CELL; LEIGH DISEASE; MALE; MEDICAL DECISION MAKING; MITOCHONDRIAL RESPIRATION; PRESCHOOL CHILD; PRIORITY JOURNAL; RESPIRATORY CHAIN;

EID: 0035666573     PISSN: 00223476     EISSN: None     Source Type: Journal    
DOI: 10.1067/mpd.2001.118885     Document Type: Article

References (14)

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3. Respiratory chain complex I deficiency: An underdiagnosed energy generation disorder
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5. Mutations in the complex I NDUFS2 gene are associated with hypertrophic cardiomyopathy and encephalomyopathy
6. Mitochondrial complex I deficiency leads to increased production of superoxide radicals and induction of superoxide dismutase
7. Riboflavin-responsive complex I deficiency
8. Therapy of mitochondrial disorders
9. Coenzyme Q10 with multiple vitamins is generally ineffective in treatment of mitochondrial disease
10. ATP synthesis in lipoamide dehydrogenase deficiency
11. The interaction of Q analogs, hydroxy decyl benzoquinone (Idebenone) with the respiratory complexes of heart mitochondria
12. Isoalloxazine ring of FAD is required for the formation of the core in the Hsp60-assisted folding of medium chain acyl-CoA dehydrogenase subunit into the assembly competent conformation in mitochondria
13. Treatment of complex I deficiency with riboflavin
14. Mitochondrial myopathy with tRNA(Leu(UUR)) mutation and complex I deficiency responsive to riboflavin