Progeria (Hutchison-Gilford syndrome) in siblings: in an autosomal recessive pattern of inheritance.

Progeria is an autosomal dominant, premature aging syndrome. Six and three year old female siblings had sclerodermatous changes over the extremities, alopecia, beaked nose, prominent veins and bird-like facies. Radiological features were consistent with features of progeria.

Stimulation of transcription and translation by aurin tricarboxylic acid in mitochondrial lysates from Ehrlich ascites cells.

We have prepared a submitochondrial fraction from Ehrlich ascites tumor cell mitochondria which shows transcription and translation activities. The antibiotic aurin tricarboxylic acid (ATA) at low concentrations induces both RNA and protein synthetic activities of the mitochondrial lysate by several fold. At high concentrations, however, ATA inhibits the translation activity but continues to stimulate the transcription activity in a dose dependent manner up to 0.

Nature of steady-state and newly synthesized mitochondrial messenger ribonucleic acids in mouse liver and Ehrlich ascites tumor cells.

The steady-state mitochondrial mRNAs in Ehrlich ascites tumor cells and mouse liver were identified by the Northern blot analysis using nick-translated mtDNA and 32P-labeled cDNA probes. The steady-state mRNA species were compared with the poly(A)-containing RNA synthesized in vitro in isolated mitoplasts. The results show that the isolated mitoplast system can efficiently transcribe almost all of the poly(A)-containing RNAs detected in the steady-state RNA population.