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Mitochondrial transcription factor A is necessary for mtDNA maintance and embryogenesis in mice
The mouse gene for mitochondrial transcription factor A (Tfam), formerly known as m-mtTFA, is disrupted by gene targetting of loxP-sites followed by cre-mediated excision in vivo and is the first mammalian protein demonstrated to regulate mtDNA copy number in vivo. Expand
Replication of animal mitochondrial DNA
Sequence and gene organization of mouse mitochondrial DNA
The mouse mitochondrial DNA genome is highly homologous in overall sequence and in gene organization to human mitochondrial DNA, with the descending order of conserved regions being tRNA genes; origin of light-strand replication; r RNA genes; knownprotein-coding genes; unidentified protein-c coding genes; displacement-loop region. Expand
Mitochondrial DNA maintenance in vertebrates.
Because features of a transcription-primed mechanism appear to be conserved in vertebrates, a general model for initiation of vertebrate heavy-strand DNA synthesis is proposed. Expand
Replication and transcription of vertebrate mitochondrial DNA.
Dilated cardiomyopathy and atrioventricular conduction blocks induced by heart-specific inactivation of mitochondrial DNA gene expression
This animal model reproduces biochemical, morphological and physiological features of the dilated cardiomyopathy of Kearns-Sayre syndrome and provides genetic evidence that the respiratory chain is critical for normal heart function. Expand
Elongation of displacement-loop strands in human and mouse mitochondrial DNA is arrested near specific template sequences.
Direct sizing at the nucleotide level indicates that the 3' ends of D-loop strands of human and mouse mtDNA are discrete and map within three to five nucleotides on the complementary template strand. Expand
Similarity of human mitochondrial transcription factor 1 to high mobility group proteins.
Human mitochondrial transcription factor 1 has been sequenced and is a nucleus-encoded DNA binding protein of 204 amino acids (24,400 daltons) with no similarities to any other DNA binding proteins except for the existence of two domains that are characteristic of high mobility group (HMG) proteins. Expand
Nuclear RNase MRP is required for correct processing of pre-5.8S rRNA in Saccharomyces cerevisiae.
Results are consistent with RNase MRP playing a role in a late step of rRNA processing and indicate a requirement for having the smaller form of 5.8S rRNA, and they argue for processing at the B1 position being composed of two separate cleavage events catalyzed by two different activities. Expand
Purification and characterization of human mitochondrial transcription factor 1.
Although mtTF1 is the only mitochondrial DNA-binding transcription factor to be purified and characterized, its properties, such as a high affinity for random DNA and a weak specificity for one of its target sequences, may typify this class of regulatory proteins. Expand