Maternal inheritance of deleted mitochondrial DNA in a family with mitochondrial myopathy

Fifteen interspecific hybrids of Serbian spruce (Piceaomorika (Panc) Purkyne) and white spruce (Piceaglauca (Moench) Voss) representing five separate crosses, including reciprocals, were used to demonstrate maternal inheritance of mitochondrial DNA. Total DNA was extracted from foliage samples of Serbian spruce (S), white spruce (W), and both S(♂) × W(♀) and W(♂) × S(♀) hybrids, digested and probed with one of two maize mitochondrial genes, ATPaseα or COXII. ATPaseα generated diagnostic Serbian and white spruce genotypes for all five enzymes tested, while COXII differentiated between the two species for four of five enzymes. Maternal inheritance was indicated in all hybrids for every diagnostic enzyme–probe combination. No paternal or nonparental bands were detected. A dilution experiment indicated that the Serbian and white spruce mitochondrial DNA restriction fragment length polymorphisms could be detected in as little as 60 and 500 ng of total DNA, respectively. It appears that the mechanism that controls the inheritance of mitochondria in Picea is still functional in wide interspecific crosses.

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Tandem direct duplications of mitochondrial DNA in mitochondrial myopathy: analysis of nucleotide sequence and tissue distribution

Nucleic Acids Research ◽

10.1093/nar/17.24.10223 ◽

1989 ◽

Vol 17 (24) ◽

pp. 10223-10229 ◽

Cited By ~ 39

Author(s):

J. Poulton ◽

M.E. Deadman ◽

R.M. Gardiner

Keyword(s):

Mitochondrial Dna ◽

Nucleotide Sequence ◽

Tissue Distribution ◽

Mitochondrial Myopathy

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Demonstration of the maternal inheritance of avian mitochondrial DNA in chicken-quail hybrids

Journal of Experimental Zoology ◽

10.1002/jez.1402360215 ◽

1985 ◽

Vol 236 (2) ◽

pp. 245-247 ◽

Cited By ~ 11

Author(s):

T. Watanabe ◽

M. Mizutani ◽

S. Wakana ◽

T. Tomita

Keyword(s):

Mitochondrial Dna ◽

Maternal Inheritance

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In vitro genetic transfer of protein synthesis and respiration defects to mitochondrial DNA-less cells with myopathy-patient mitochondria.

Molecular and Cellular Biology ◽

10.1128/mcb.11.4.2236 ◽

1991 ◽

Vol 11 (4) ◽

pp. 2236-2244 ◽

Cited By ~ 205

Author(s):

A Chomyn ◽

G Meola ◽

N Bresolin ◽

S T Lai ◽

G Scarlato ◽

...

Keyword(s):

Mitochondrial Dna ◽

Protein Synthesis ◽

Human Cell ◽

Mitochondrial Myopathy ◽

Mitochondrial Protein ◽

Human Cell Lines ◽

Mitochondrial Protein Synthesis ◽

Direct Link ◽

Genetic Transfer

A severe mitochondrial protein synthesis defect in myoblasts from a patient with mitochondrial myopathy was transferred with myoblast mitochondria into two genetically unrelated mitochondrial DNA (mtDNA)-less human cell lines, pointing to an mtDNA alteration as being responsible and sufficient for causing the disease. The transfer of the defect correlated with marked deficiencies in respiration and cytochrome c oxidase activity of the transformants and the presence in their mitochondria of mtDNA carrying a tRNA(Lys) mutation. Furthermore, apparently complete segregation of the defective genotype and phenotype was observed in the transformants derived from the heterogeneous proband myoblast population, suggesting that the mtDNA heteroplasmy in this population was to a large extent intercellular. The present work thus establishes a direct link between mtDNA alteration and a biochemical defect.

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