Expression of mtDNA and nDNA encoded respiratory chain proteins in chemically and genetically-derived Rho0 human fibroblasts: a comparison of subunit proteins in normal fibroblasts treated with ethidium bromide and fibroblasts from a patient with mtDNA depletion syndrome

The Whole Structure of the 13-Subunit Oxidized Cytochrome c Oxidase at 2.8 Å

1996 • 2,089 citations

Human cells lacking mtDNA: repopulation with exogenous mitochondria by complementation

1990 • 1,390 citations

DISEASES OF THE MITOCHONDRIAL DNA

1992 • 1,294 citations

Cytochrome oxidase: an endogenous metabolic marker for neuronal activity

1989 • 1,231 citations

A new mitochondrial disease associated with mitochondrial DNA heteroplasmy.

1990 • 981 citations

mtDNA depletion with variable tissue expression: a novel genetic abnormality in mitochondrial diseases.

1991 • 588 citations

Mitochondrial DNA sequence variation in human evolution and disease.

1994 • 526 citations

MELAS mutation in mtDNA binding site for transcription termination factor causes defects in protein synthesis and in respiration but no change in levels of upstream and downstream mature transcripts.

1992 • 490 citations

Mitochondrial DNA and Disease

1995 • 444 citations

[2] Citrate synthase from rat liver

1969 • 435 citations

NRF-1: a trans-activator of nuclear-encoded respiratory genes in animal cells.

1990 • 404 citations

Mitochondrial Encephalomyopathies

1993 • 297 citations

Interaction of Nuclear Factors with Multiple Sites in the Somatic Cytochrome c Promoter

1989 • 259 citations

Benign infantile mitochondrial myopathy due to reversible cytochrome c oxidase deficiency

1983 • 231 citations

Mitochondrial myopathy of childhood associated with depletion of mitochondrial DNA

1992 • 214 citations

Detection of "deleted" mitochondrial genomes in cytochrome-c oxidase-deficient muscle fibers of a patient with Kearns-Sayre syndrome.

1989 • 212 citations

Deficiency of the human mitochondrial transcription factor h-mtTFA in infantile mitochondrial myopathy is associated with mtDNA depletion

1994 • 170 citations

[9] Mammalian cytochrome-c oxidase: Characterization of enzyme and immunological detection of subunits in tissue extracts and whole cells

1995 • 138 citations

Complementation and segregation behavior of disease-causing mitochondrial DNA mutations in cellular model systems

1995 • 132 citations

Nuclear complementation restores mtDNA levels in cultured cells from a patient with mtDNA depletion.

1993 • 117 citations

Cytochrome c Oxidase Deficiency

1990 • 112 citations

Fatal infantile liver failure associated with mitochondrial DNA depletion

1992 • 111 citations

Depletion of mitochondrial deoxyribonucleic acid in a family with fatal neonatal liver disease

1996 • 86 citations

Mitochondrial myopathies and respiratory chain proteins

1988 • 78 citations

Investigation of mitochondrial metabolism in small human skeletal muscle biopsy specimens. Improvement of preparation procedure

1985 • 71 citations

Lacticacidemia

1993 • 69 citations

Mitochondrial DNA depletion: Prevalence in a pediatric population referred for neurologic evaluation

1996 • 68 citations

The rat cytochromecoxidase subunit IV gene family: tissue-specific and hormonal differences in subunit IV and cytochromecmRNA expression

1990 • 66 citations

Depletion of mitochondrial DNA in the liver of a patient with lactic acidemia and hypoketotic hypoglycemia

1996 • 63 citations

Subunit specific monoclonal antibodies show different steady-state levels of various cytochrome-c oxidase subunits in chronic progressive external ophthalmoplegia

1996 • 57 citations

Mitochondria in cultured human muscle cells depleted of mitochondrial DNA.

1993 • 55 citations

Tissue distribution of cytochrome c oxidase isoforms in mammals. Characterization with monoclonal and polyclonal antibodies

1993 • 50 citations

Early-onset encephalomyopathy associated with tissue-specific mitochondrial DNA depletion: A morphological, biochemical and molecular-genetic study

1995 • 49 citations

Expression and fate of the nuclearly encoded subunits of cytochrome-c oxidase in cultured human cells depleted of mitochondrial gene products

1995 • 43 citations

Determination of the structures of respiratory enzyme complexes from mammalian mitochondria

1995 • 42 citations

Efficient hybridoma production using previously frozen splenocytes

1988 • 42 citations

Respiratory-deficient human fibroblasts exhibiting defective mitochondrial DNA replication

1995 • 35 citations

Disorders Associated with Depletion of Mitochondrial DNA

1992 • 33 citations

Fatal cytochromec oxidase‐deficient myopathy of infancy associated with mtDNA depletion. Differential involvement of skeletal muscle and cultured fibroblasts

1992 • 30 citations

Early-onset fatal encephalomyopathy associated with severe mtDNA depletion

1995 • 25 citations

An Antisense Oligodeoxynucleotide Approach to Investigate the Function of the Nuclear-Encoded Subunits of Human Cytochrome c Oxidase

1993 • 17 citations

Molecular Cloning and Characterization of the Rat Cytochrome c Oxidase Subunit Vb Gene

1994 • 10 citations