Osmoregulation in Polycystic Kidney Disease: Relationship with Cystogenesis and Hypertension
نویسندگان
چکیده
منابع مشابه
New Therapies Targeting Cystogenesis in Autosomal Polycystic Kidney Disease
Autosomal dominant polycystic kidney disease is the most common inherited kidney disease and results from mutations in the polycystin 1 gene (PKD1) or the polycystin 2 gene (PKD2). The disease is characterised by the progressive development of fluid-filled cysts derived from renal tubular epithelial cells that destroy the architecture of the renal parenchyma and lead to kidney failure. Until re...
متن کاملAutosomal dominant polycystic kidney disease is associated with central and nephrogenic defects in osmoregulation.
Autosomal dominant polycystic kidney disease (ADPKD) is associated with a urine-concentrating defect attributed to renal cystic changes. As PKD genes are expressed in the brain, altered central release of arginine vasopressin could also play a role. In order to help determine this we measured central and nephrogenic components of osmoregulation in 10 adults and 10 children with ADPKD, all with ...
متن کاملTransepithelial chloride secretion and cystogenesis in autosomal dominant polycystic kidney disease.
Despite these advances in the genetics of ADPKD, Autosomal dominant polycystic kidney disease the link between the mutated polycystins and the (ADPKD) is one of the most common monogenic cystogenesis process remains largely speculative. hereditary diseases (prevalence 15400–151000). It is Observations in PKD1 −/− mice indicate that characterized by the development of multiple cysts in polycysti...
متن کاملPax2 gene dosage influences cystogenesis in autosomal dominant polycystic kidney disease.
Mutations in PKD1 cause dominant polycystic kidney disease (PKD), characterized by large fluid-filled kidney cysts in adult life, but the molecular mechanism of cystogenesis remains obscure. Ostrom et al. [Dev. Biol., 219, 250-258 (2000)] showed that reduced dosage of Pax2 caused increased apoptosis, and ameliorated cystogenesis in Cpk mutant mice with recessive PKD. Pax2 is expressed in conden...
متن کاملGenetic evidence for a trans-heterozygous model for cystogenesis in autosomal dominant polycystic kidney disease.
Polycystic kidney disease (ADPKD) is a condition with an autosomal dominant mode of inheritance and adult onset. Two forms of the disease, ADPKD1 and ADPKD2, caused by mutations in PKD1 and PKD2, respectively, are very similar, except that ADPKD1 patients run a more severe course. At the cellular level, ADPKD1 was first shown to be recessive, since somatic second hits are perhaps necessary for ...
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ژورنال
عنوان ژورنال: Annals of Nutrition and Metabolism
سال: 2018
ISSN: 0250-6807,1421-9697
DOI: 10.1159/000488125