[Elevated expression of argentophilic proteins from the nucleolar organizer regions in myocardium of patients with obstructive hypertrophic cardiomyopathy and mutations in p53 tumor suppressor gene].
Silver staining for nucleolar proteins was used to evaluate the ribosomal genes activity in cardiomyocytes (Cm) and fibroblast-like cells (FBS) of intraventricular septum, and other regions of the left ventricle. Specimens to be analysed were taken from 7 patients with idiopathic obstructive hypertrophic cardiomyopathy (OHCM). In this group the quantity of nucleoli and AgNORs, reflecting the transcriptional and processing level of pre-ribosomal RNA in all type of cells, was at least 2 and 3 times higher than in patients with essential hypertension and in healthy control, respectively. We suggest that nucleolar hypertrophy in patients with hypertrophic cardiomyopathies, inappropriate to hypertrophy in other pathological conditions, may be most probably of compensatory character, and this may be in part explained by nuclear hyperploidy and chromosomal endoreduplication. We have noted a marked heterogeneity in shape, size and quantity of nucleoli, and in AgNORs of FBC. Nuclei with modulated phenotype containing nucleoli of high activity were revealed. This article presents the first data on p53 mutation identification in patients with advanced OHCM.
Gudkova
A Ia
AIa
Pavlov St. Petersburg State Medical University.
Shliakhto
E V
EV
Mamaev
N N
NN
Rybakova
M G
MG
Amineva
Kh K
KhK
Semernin
E N
EN
Korzhenevskaia
K V
KV
Zaraĭskiĭ
M I
MI
D000328:Adult; D002312:Cardiomyopathy, Hypertrophic / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D009154:Mutation; D009206:Myocardium / Q000378:metabolism* / Q000648:ultrastructure; D009687:Nuclear Proteins / Q000096:biosynthesis*; D009697:Nucleolus Organizer Region / Q000378:metabolism* / Q000648:ultrastructure; D016622:Silver Staining; D016159:Tumor Suppressor Protein p53 / Q000235:genetics*
D009687:Nuclear Proteins; D016159:Tumor Suppressor Protein p53