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J Biol Chem, Vol. 274, Issue 47, 33426-33432, November 19, 1999
From INSERM-EMI 9929, Université Victor Segalen-Bordeaux 2, 146 rue Léo-Saignat, F-33076 Bordeaux Cedex, France
Mitochondrial cytopathies present a tissue
specificity characterized by the fact that even if a mitochondrial DNA
mutation is present in all tissues, only some will be affected and
induce a pathology. Several mechanisms have been proposed to explain this phenomenon such as the appearance of a sporadic mutation in a
given stem cell during embryogenesis or mitotic segregation, giving
different degrees of heteroplasmy in tissues. However, these mechanisms
cannot be the only ones involved in tissue specificity. In this paper,
we propose an additional mechanism contributing to tissue specificity.
It is based on the metabolic expression of the defect in oxidative
phosphorylation (OXPHOS) complexes that can present a biochemical
threshold. The value of this threshold for a given OXPHOS complex can
vary according to the tissue; thus different tissues will display
different sensitivities to a defect in an OXPHOS complex. To verify
this hypothesis and to illustrate the pathological consequences of the
variation in biochemical thresholds, we studied their values for seven
OXPHOS complexes in mitochondria isolated from five different rat
tissues. Two types of behavior in the threshold curves can be
distinguished corresponding to two modes of OXPHOS response to a
deficiency. We propose a classification of tissues according to their
type of OXPHOS response to a complex deficiency and therefore to their threshold values.
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