Abstract
Mitochondrial disorders encompass any medical specialty and affect patients at any age. Likewise, the spectrum of clinical and genetic signatures of these disorders is ample, making a precise diagnosis difficult. We will report some of the major clinical phenotypes observed in infancy, their underlining molecular features, and will propose an approach to reach a more complete diagnosis.
MeSH terms
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Brain / metabolism
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Brain / pathology
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Coenzymes / deficiency
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DNA, Mitochondrial / genetics
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Electron Transport Complex I / deficiency
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Electron Transport Complex I / genetics
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Humans
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Infant
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Leigh Disease / genetics
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Leigh Disease / metabolism
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Leigh Disease / pathology
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Mitochondrial Diseases / genetics
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Mitochondrial Diseases / metabolism
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Mitochondrial Diseases / pathology*
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Mutation*
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Succinate-CoA Ligases / deficiency
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Succinate-CoA Ligases / genetics
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Ubiquinone / analogs & derivatives*
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Ubiquinone / deficiency
Substances
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Coenzymes
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DNA, Mitochondrial
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Ubiquinone
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Succinate-CoA Ligases
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SUCLA2 protein, human
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Electron Transport Complex I
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coenzyme Q10