Tissular distribution of heteroplasmy and ultrastructural studies of mitochondria from a <i>Drosophila subobscura</i> mitochondrial deletion mutant

Lécher, Pierre; Beziat, Florence; Alziari, Serge

doi:10.1016/0248-4900(94)90013-2

Cited by 18 publications

(19 citation statements)

References 17 publications

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“…On the other hand, in fractions enriched with germ cells, namely male or female abdomens, heteroplasmy is slightly lower (respectively, 71% and 63%). The germ line is apparently less rich in deleted mtDNA, as observed previously (15). These results indicate that the deleted molecules are already present in germ cells, as has been shown in man (29).…”

Section: Discussionsupporting

confidence: 73%

“…Results thus bear out those observed for the whole fly (14): mutations affecting complex I and complex III activities do not affect complex IV activity. This indicates that in the mutant strain, the protein synthesis in all mitochondria is normal, as observed previously (15).…”

Section: Comparison Of Biochemical Activities In Fractions Of the Mutsupporting

confidence: 66%

“…Nevertheless, given the specific transcription system for mitochondrial genes (31), mtDNA Deletion Consequences on D. subobscura Mutant Strainthe degradation kinetics are probably modified. This might be due to increased protection by mitoribosomes, which show identical concentrations in the two strains (15). Detection of the fusion transcript in all studied fractions indicates general expression of the deleted genomes.…”

Section: Discussionmentioning

confidence: 96%

“…Moreover, cytochrome oxidase activity was detectable in all mitochondria. Heteroplasmy was probably intramitochondrial (15,16), since both types of genomes coexisted in each mitochondria.…”

mentioning

confidence: 99%

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Biochemical and Molecular Consequences of Massive Mitochondrial Gene Loss in Different Tissues of a Mutant Strain of Drosophila subobscura

Beziat

Touraille

Debise

et al. 1997

Journal of Biological Chemistry

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In the studied mutant strain of Drosophila subobscura, 78% of the mitochondrial genomes lost >30% of the coding region by deletion. The mutations was genetically stable. Despite this massive loss of mitochondrial genes, the mutant did not seem to be affected. Distribution of the two genome types, cell levels of mitochondrial DNA, steady-state concentrations of the mitochondrial gene transcripts, mitochondrial enzymatic activities, and ATP synthesis capacities were measured in the head, thorax, and abdomen fractions of the mutant strain in comparison with a wild type strain. Results indicate that the deleted genomes are detected in all fractions but to a lesser extent in the male and female abdomen. In all fractions, there is a 50% increase in cellular mitochondrial DNA content. Although there is a decrease in steady-state concentrations of mitochondrial transcripts of genes affected by deletion, this is smaller than expected. The variations in mitochondrial biochemical activities in the different fractions of the wild strain are upheld in the mutant strain. Activity of complex I (involved in mutation) nevertheless shows a decrease in all fractions; activity of complex III (likewise involved) shows little or no change; finally, mitochondrial ATP synthesis capacity is identical to that observed in the wild strain. This latter finding possibly accounts for the lack of phenotype. This mutant is a good model for studying mitochondrial genome alterations and the role of the nuclear genome in these phenomena.Various alterations in the mitochondrial genome have been correlated with severe human pathology (1-3). Among the most substantial of these alterations, deletions, which account for a small fraction of the described cases, have always been detected at the heteroplasmic state (4 -6). Once the proportion of deleted genomes reaches a critical threshold, these deletions have often very pejorative effects on mitochondrial function (7,8). Such mutations are generally sporadic, although dominant autosomal factors have been observed (9 -11). A locus has been localized on chromosome 10, but no gene has yet been identified.In the heteroplasmic state, the mutant Drosophila subobscura strain studied in our laboratory presents a substantial mtDNA 1 deletion (30% of the coding region) (12). Contrary to clinical findings (1), this mutation remains stable from one generation to the next. It does not seem to affect the mutant (apparent absence of phenotype) in terms of reproduction (number of embryos, emergence of larvae), life span, or flight capacities. This strain thus offers a good model for studying possible biochemical and molecular consequences of severe mitochondrial genome alteration and the responses that enable the mutant to survive.The first investigations, performed in the whole adult fly (13), showed a strong heteroplasmic tendency in the deleted molecules; this affected 78% of the mitochondrial genomes. mtDNA cell content (per nuclear genome) showed a 50% increase in the mutant strain compared with the D. subobscura ...

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Section: Discussionsupporting

confidence: 73%

Section: Comparison Of Biochemical Activities In Fractions Of the Mutsupporting

confidence: 66%

Section: Discussionmentioning

confidence: 96%

“…Moreover, cytochrome oxidase activity was detectable in all mitochondria. Heteroplasmy was probably intramitochondrial (15,16), since both types of genomes coexisted in each mitochondria.…”

mentioning

confidence: 99%

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Biochemical and Molecular Consequences of Massive Mitochondrial Gene Loss in Different Tissues of a Mutant Strain of Drosophila subobscura

Beziat

Touraille

Debise

et al. 1997

Journal of Biological Chemistry

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show abstract

“…It is widely accepted that the distribution of different ratios of mutant to wt-mtDNA in different tissues is important in determining the clinical phenotype. Generally, heteroplasmy is known to be intracellular, and several reports suggest that heteroplasmy is intra-mitochondrial, in both humans and Drosophila (Lécher et al 1994;Attardi et al 1995;Matthews et al 1995).…”

Section: Introductionmentioning

confidence: 99%

Preferential amplification and phenotypic selection in a population of deleted and wild-type mitochondrial DNA in cultured cells

et al. 1997

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A Heteroplasmic Strain of D. Subobscura. An Animal Model of Mitochondrial Genome Rearrangement

Alziari

Petit

Lefai

et al. 1999

Mitochondrial Diseases

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Tissular distribution of heteroplasmy and ultrastructural studies of mitochondria from a Drosophila subobscura mitochondrial deletion mutant

Cited by 18 publications

References 17 publications

Biochemical and Molecular Consequences of Massive Mitochondrial Gene Loss in Different Tissues of a Mutant Strain of Drosophila subobscura

Biochemical and Molecular Consequences of Massive Mitochondrial Gene Loss in Different Tissues of a Mutant Strain of Drosophila subobscura

Preferential amplification and phenotypic selection in a population of deleted and wild-type mitochondrial DNA in cultured cells

A Heteroplasmic Strain of D. Subobscura. An Animal Model of Mitochondrial Genome Rearrangement

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