Infantile mitochondrial disorders

Biosci Rep. 2007 Jun;27(1-3):105-12. doi: 10.1007/s10540-007-9039-y.

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.

Publication types

  • Review

MeSH terms

  • Brain / metabolism
  • Brain / pathology
  • Coenzymes / deficiency
  • DNA, Mitochondrial / genetics
  • Electron Transport Complex I / deficiency
  • Electron Transport Complex I / genetics
  • Humans
  • Infant
  • Leigh Disease / genetics
  • Leigh Disease / metabolism
  • Leigh Disease / pathology
  • Mitochondrial Diseases / genetics
  • Mitochondrial Diseases / metabolism
  • Mitochondrial Diseases / pathology*
  • Mutation*
  • Succinate-CoA Ligases / deficiency
  • Succinate-CoA Ligases / genetics
  • Ubiquinone / analogs & derivatives*
  • Ubiquinone / deficiency

Substances

  • Coenzymes
  • DNA, Mitochondrial
  • Ubiquinone
  • Succinate-CoA Ligases
  • SUCLA2 protein, human
  • Electron Transport Complex I
  • coenzyme Q10