Аннотация:
Polycystic kidney disease (PKD) is a genetically determined pathological process associated with the formation and growth of cysts originating from the epithelial cells of the tubules and/or collecting tubes. PBP is represented by two main types - autosomal dominant (ADPKD) and autosomal recessive PKD (ARPKD), which are different diseases. The main causes of ADPKD are mutations of the PKD1 and PKD2 genes, which encode the formation of polycystin-1 and polycystin-2 proteins. ARPKD-linked mutation in the gene PKHD1, leads to total absence or defective synthesis of receptor protein primary cilia - fibrocystin. There are relationships between the structural and functional defects in the primary cilia and PBP. Mechanisms of cysts formation and growth include a) mutations of polycystines genes located on the cilia; b) increased activity of renal intracellular cAMP; c) vasopressin V2 receptors activation; d) violation of the tubular epithelium polarity (translocation of Na,K-ATPasa from basol
Руденко Т. Е. Татьяна Евгеньевна 1975-
Бобкова И. Н. Ирина Николаевна 1967-
Ставровская Е. В. Екатерина Викторовна 1970-
Rudenko T. E. Tat`yana Evgenyevna 1975-
Bobkova I. N. Irina Nikolaevna 1967-
Stavrovskaya E. V. Ekaterina Viktorovna 1970-
Modern approaches to conservative therapy of polycystic kidney disease
Текст визуальный непосредственный
Терапевтический архив
Медицинское маркетинговое агентство "МедиаМедика"
Т. 91, Вып. 6 С. 116-123
2019
Статья
CAMP Fibrocystin MTOR inhibitors Polycystic kidney disease Polycystin 1 and 2 Somatostatin analogues Vasopressin receptor V2 antagonists
Polycystic kidney disease (PKD) is a genetically determined pathological process associated with the formation and growth of cysts originating from the epithelial cells of the tubules and/or collecting tubes. PBP is represented by two main types - autosomal dominant (ADPKD) and autosomal recessive PKD (ARPKD), which are different diseases. The main causes of ADPKD are mutations of the PKD1 and PKD2 genes, which encode the formation of polycystin-1 and polycystin-2 proteins. ARPKD-linked mutation in the gene PKHD1, leads to total absence or defective synthesis of receptor protein primary cilia - fibrocystin. There are relationships between the structural and functional defects in the primary cilia and PBP. Mechanisms of cysts formation and growth include a) mutations of polycystines genes located on the cilia; b) increased activity of renal intracellular cAMP; c) vasopressin V2 receptors activation; d) violation of the tubular epithelium polarity (translocation of Na,K-ATPasa from basol