Electrophoretic profiles of nonhistone nuclear proteins of human hearts with muscular subaortic stenosis.

Abstract

SUMMARY Muscular subaortic stenosis (MSS) is a genetically determined cardiomyopathy, whereas right ventricular infundibular hypertrophy (IH) apparently is an acquired condition. Since genetic expression in eukaryotic cells may be regulated primarily by DNA-associated proteins, we isolated and characterized the proteins of heart nuclei from nine patients with MSS, eight with IH, and two with normal (N) hearts. More than 150 proteins could be identified by two-dimensional polyacrylamide gel electrophoresis. Proteins in the entire region from pH 7.0 to 9.0 with molecular weights (M r ) ranging from 35,000 to 41,000 and a protein focusing from pH 5.2 to 5.3 with M r of 55,000 were strikingly reduced in MSS. Again the electrophoretic patterns of N and IH were similar. The electrophoretic patterns of nonhistone nuclear protein (NHNP) in MSS relative to N showed a striking resemblance to those demonstrated previously for the early stage (myolytic phase) of hamster cardiomyopathy relative to the matched control. Since NHNP interacting with DNA appears to play a major role in genetic expression, it is possible that some of the manifestations of MSS could be due to different components of NHNP in the affected hearts. Circ Res 46: 513-519, 1980

The nuclear proteins in the liver of rabbits with an inherited motorial failure

“…The influence of genetically determined pathological states on the composition of nonhistone proteins was observed in relation to cardiomyopatic syrian hamsters (L iew and S ole 1978) and the muscular subaortic stenosis of the human heart (L iew et al 1980). In all these cases the disappearance of a group of nuclear proteins with molecular weights between 35000 and 41000 D and isoelectric points between 7 and 9 was observed.…”

Changes in non-histone nuclear proteins during postnatal myocardial development

“…In the last few years we have been involved in identifying and characterizing DNA-associated proteins in various tissues by two-dimensional polyacrylamide-gel electrophoresis (Suria & Liew, 1974a;Liew & Chan, 1976;Chan & Liew, 1977, 1979Jackowski et al, 1976;Liew, 1979). We have also employed this technique to identify myocardial nuclear protein changes associated with genetically determined cardiomyopathy in the Syrian hamster and in the human (Liew & Sole, 1978a,b;Liew et al, 1980). By using this technique, we proceeded to investigate age-related changes in specific myocardial nuclear proteins during postnatal development.…”