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Deficiency of subunits in heart mitochondrial NADH-ubiquinone oxidoreductase of a patient with mitochondrial encephalomyopathy and cardiomyopathy
- Source :
- Biochemical and Biophysical Research Communications. 140:88-93
- Publication Year :
- 1986
- Publisher :
- Elsevier BV, 1986.
-
Abstract
- The heart mitochondria isolated from a patient with hypertrophic cardiomyopathy associated with mitochondrial encephalomyopathy were analyzed by immunoblotting using specific antibody against each of the purified mitochondrial energy transducing complexes from beef heart. Subunits of NADH-ubiquinone oxidoreductase (Complex I) were markedly decreased and those of cytochrome c oxidase (Complex IV) were decreased to some extent, but the deficiency of any of these subunits was only partial. On the other hand, the contents of subunits of ubiquinol-cytochrome c oxidoreductase (Complex III) were normal. These results suggest that the decreased levels of some of the Complex I subunits might be the primary cause of disorder in this patient.
- Subjects :
- Mitochondrial encephalomyopathy
Adolescent
Biophysics
Cardiomyopathy
Cytochrome-c Oxidase Deficiency
Biology
Mitochondrion
Biochemistry
Mitochondria, Heart
Muscular Diseases
Oxidoreductase
NAD(P)H Dehydrogenase (Quinone)
medicine
Humans
Cytochrome c oxidase
Quinone Reductases
Molecular Biology
chemistry.chemical_classification
NADH-Ubiquinone Oxidoreductase
Brain Diseases, Metabolic
Hypertrophic cardiomyopathy
Cell Biology
Cardiomyopathy, Hypertrophic
medicine.disease
Molecular biology
chemistry
Coenzyme Q – cytochrome c reductase
biology.protein
Female
Subjects
Details
- ISSN :
- 0006291X
- Volume :
- 140
- Database :
- OpenAIRE
- Journal :
- Biochemical and Biophysical Research Communications
- Accession number :
- edsair.doi.dedup.....10d661f5521d5172dcac05a7d1574282